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1. A combinatorial flow-based formulation for temporal bin packing problems

Author: Martinovic, J., Strasdat, N., Valério de Carvalho, J., and Furini, F.
Published: 2023
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2. Factors associated with fertility abnormalities in women with systemic lupus erythematosus: a systematic review and meta-analysis

Author: Giambalvo, S., Garaffoni, C., Silvagni, E., Furini, F., Rizzo, R., Govoni, M., and Bortoluzzi, A.
Published: 2022
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3. Predictors of disease activity in gout: a 12-month analysis of the ATTACk (Achieving improvement in the management of crystal-induced arthritis) multicentre cohort study

Author: Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Bortoluzzi, A, Ceccarelli, F, Lucchetti, R, Furini, F, Del Ross, T, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Bortoluzzi A., Ceccarelli F., Lucchetti R., Furini F., Del Ross T., Zanetti A., Carrara G., Scire C. A., Doria A., Ramonda R., Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Bortoluzzi, A, Ceccarelli, F, Lucchetti, R, Furini, F, Del Ross, T, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Bortoluzzi A., Ceccarelli F., Lucchetti R., Furini F., Del Ross T., Zanetti A., Carrara G., Scire C. A., Doria A., and Ramonda R.
Abstract: Objective Gout treatment is largely suboptimal in clinical practice. We aimed to assess the predictors of disease-activity at 12 months in a real-life setting. Methods Consecutive patients referred to Rheumatology Units for suspected acute crystal-induced arthritis were enrolled in a multicentre-cohort study. Only patients with clinical diagnosis of gout were eligible. Disease-activity was evaluated by the Patient Acceptable Symptom State (PASS) on a visual analogue scale (VAS, 0=unsatisfactory, 100=satisfactory) at 0 (T0) and 12 months (T12), and the composite score called Gout Activity Score (GAS) calculated on the number of arthritic attacks (flare count), serum uric acid (sUA), cumulative number of tophi, VAS (T12), PtGA (T12). Multivariate linear regression model was performed to assess predictors of gout disease-activity at T12 with PASS and GAS as outcomes. Results: 201 patients had gout (diagnosis on synovial fluid in 45%, tophi in 26%, mean sUA 7.4±1.9 mg/L, 85% with urate-lowering therapy (ULT) in progress/initiated at T0); mean age 63±13 years, 88% men, median (interquartile range) disease duration 2.9 years (0.7-9.4). Follow-up visits were performed in 113 (56%) patients at T12. Mean PASS observed at T0 and at T12 were 38±27 and 74±23, respectively, whereas GAS at T12 was 10±8. A significant association was observed between the presence of tophi and PASS at T12 (-15.3, 95% CI -25.5, -5.2; p=0.003) and GAS at T12 (+4.0, 95% CI 0.6,7.4; p=0.02), adjusted for age, sex, disease duration, sUA <6 mg/dL, tender joint count, PASS at T0, ULT). CONCLUSIONS:The baseline presence of tophi may predict high disease-activity at T12, thus worsening GAS and patients’ pain perception.
Published: 2023

4. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author: De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., and Rossella T.
Abstract: Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.
Published: 2023

5. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author: De Angelis R., Ferri C., Giuggioli D., Bajocchi G., Dagna L., Bellando-Randone S., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Lepri G., Girelli F., Riccieri V., Zanatta E., Bosello S. L., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A. M., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Cipolletta E., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Di Vico C., Gigante A., Pellagrino G., Pigatto E., Lazzaroni M. -G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Guiducci S., Doria A., Salvarani C., Iannone F., Matucci-Cerinic M., Giorgio A., Alessia B., Francesca C., Renato C., Dall'Ara F., Angelo D. C., Marica D., Gianluca S., Rossella T., De Angelis, R, Ferri, C, Giuggioli, D, Bajocchi, G, Dagna, L, Bellando-Randone, S, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Lepri, G, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Cipolletta, E, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Di Vico, C, Gigante, A, Pellagrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Guiducci, S, Doria, A, Salvarani, C, Iannone, F, Matucci-Cerinic, M, Giorgio, A, Alessia, B, Francesca, C, Renato, C, Dall'Ara, F, Angelo, D, Marica, D, Gianluca, S, Rossella, T, De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, S. L., Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, E., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, G. D., Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., and Rossella, T.
Subjects: Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, Epidemiology, Immunology, Systemic, Autoimmunity, Autoimmune Disease, Autoimmune Diseases, Scleroderma, Rheumatology, Immunology and Allergy, Seasons, Human
Abstract: ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.ResultsAmong patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (pConclusionThe ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.
Published: 2023

6. Impact of disease duration and gender on the sensitivity and specificity of 2015 ACR/EULAR classification criteria for gout. Cross-sectional results from an Italian multicentric study on the management of crystal-induced arthritis (ATTACk)

Author: Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Ceccarelli, F, Lucchetti, R, Bortoluzzi, A, Cimmino, M, Di Matteo, A, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Furini, F, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Ceccarelli F., Lucchetti R., Bortoluzzi A., Cimmino M. A., Di Matteo A., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Furini F., Zanetti A., Carrara G., Scire C. A., Doria A., Ramonda R., Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Ceccarelli, F, Lucchetti, R, Bortoluzzi, A, Cimmino, M, Di Matteo, A, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Furini, F, Zanetti, A, Carrara, G, Scire, C, Doria, A, Ramonda, R, Lorenzin M., Ughi N., Ariani A., Raffeiner B., Ceccarelli F., Lucchetti R., Bortoluzzi A., Cimmino M. A., Di Matteo A., Frallonardo P., Hoxha A., Ortolan A., Favero M., Parisi S., Furini F., Zanetti A., Carrara G., Scire C. A., Doria A., and Ramonda R.
Abstract: Objective We aimed to assess the performance of the 2015 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) gout classification criteria in an Italian cohort of patients with crystal-induced arthritis stratified by disease duration and gender in a real-life setting. Methods Consecutive patients referred to Rheumatology Units for suspected acute crystal-induced arthritis were enrolled in a multicentre cohort study by the Italian Society of Rheumatology which was designed to improve the management of crystal-induced arthritis (ATTACk). To test the performance of the criteria (sensitivity and specificity), the presence of monosodium urate (MSU) crystals in synovial fluid (SF) was used as gold standard. Subgroup analyses by gender and disease duration were performed. Results Two hundred and seventy-seven patients were enrolled. SF analysis was available in 137 (49%) patients. Complete SF analysis and ACR/EULAR scores were obtained in 44% of patients. MSU crystals were found in 66% of patients. The sensitivity and the specificity of all criteria sets were 78% (95%CI, 67–86) and 98% (95%CI, 87–100), respectively; only clinical criteria yielded 70% (95%CI, 59–80) sensitivity and 93% (95%CI, 80-98) specificity, respectively. In early-stage disease (<2 years), the sensitivity dropped to 58% (95%CI, 39-75), while the specificity was 100% (95%CI, 85–100). Conclusion The ACR/EULAR criteria showed good performance in patients presenting with acute arthritis; changes were observed when a subset of criteria were used, especially in early-stage disease.
Published: 2022

7. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Author: Ferri, C, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri C., De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Lazzaroni M. G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., Matucci-Cerinic M., Ferri, C, De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, De Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Lazzaroni, M, Franceschini, F, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri C., De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., De Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Lazzaroni M. G., Franceschini F., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., and Matucci-Cerinic M.
Abstract: Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature. Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas. Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches. Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.
Published: 2022

8. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author: De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Dall'Ara F., Lazzaroni M. G., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Risa A. M., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., Matucci-Cerinic M., Ferri C., De Angelis, R, Giuggioli, D, Bajocchi, G, Dagna, L, Zanframundo, G, Foti, R, Cacciapaglia, F, Cuomo, G, Ariani, A, Rosato, E, Guiducci, S, Girelli, F, Riccieri, V, Zanatta, E, Bosello, S, Cavazzana, I, Ingegnoli, F, Santis, M, Murdaca, G, Abignano, G, Romeo, N, Della Rossa, A, Caminiti, M, Iuliano, A, Ciano, G, Beretta, L, Bagnato, G, Lubrano, E, De Andres, I, Giollo, A, Saracco, M, Agnes, C, Lumetti, F, Spinella, A, Magnani, L, Campochiaro, C, De Luca, G, Codullo, V, Visalli, E, Masini, F, Gigante, A, Bellando-Randone, S, Pellegrino, G, Pigatto, E, Dall'Ara, F, Lazzaroni, M, Generali, E, Mennillo, G, Barsotti, S, Mariano, G, Calabrese, F, Furini, F, Vultaggio, L, Parisi, S, Peroni, C, Risa, A, Rozza, D, Zanetti, A, Carrara, G, Landolfi, G, Scire, C, Bianchi, G, Fusaro, E, Sebastiani, G, Govoni, M, D'Angelo, S, Cozzi, F, Doria, A, Iannone, F, Salvarani, C, Matucci-Cerinic, M, Ferri, C, De Angelis R., Giuggioli D., Bajocchi G., Dagna L., Zanframundo G., Foti R., Cacciapaglia F., Cuomo G., Ariani A., Rosato E., Guiducci S., Girelli F., Riccieri V., Zanatta E., Bosello S., Cavazzana I., Ingegnoli F., Santis M., Murdaca G., Abignano G., Romeo N., Della Rossa A., Caminiti M., Iuliano A., Ciano G., Beretta L., Bagnato G., Lubrano E., De Andres I., Giollo A., Saracco M., Agnes C., Lumetti F., Spinella A., Magnani L., Campochiaro C., De Luca G., Codullo V., Visalli E., Masini F., Gigante A., Bellando-Randone S., Pellegrino G., Pigatto E., Dall'Ara F., Lazzaroni M. G., Generali E., Mennillo G., Barsotti S., Mariano G. P., Calabrese F., Furini F., Vultaggio L., Parisi S., Peroni C. L., Risa A. M., Rozza D., Zanetti A., Carrara G., Landolfi G., Scire C. A., Bianchi G., Fusaro E., Sebastiani G. D., Govoni M., D'Angelo S., Cozzi F., Doria A., Iannone F., Salvarani C., Matucci-Cerinic M., and Ferri C.
Abstract: OBJECTIVE: There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics. METHODS: A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared. RESULTS: The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs. CONCLUSION: Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.
Published: 2022

9. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author: Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.
Abstract: Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.
Published: 2022

10. Relevant domains and outcome measurement instruments in neuropsychiatric systemic lupus erythematosus: a systematic literature review

Author: Silvagni, E, Chessa, E, Bergossi, F, D'Amico, M, Furini, F, Guerrini, G, Cauli, A, Scire, C, Bertsias, G, Govoni, M, Piga, M, Bortoluzzi, A, Silvagni E., Chessa E., Bergossi F., D'Amico M. E., Furini F., Guerrini G., Cauli A., Scire C. A., Bertsias G., Govoni M., Piga M., Bortoluzzi A., Silvagni, E, Chessa, E, Bergossi, F, D'Amico, M, Furini, F, Guerrini, G, Cauli, A, Scire, C, Bertsias, G, Govoni, M, Piga, M, Bortoluzzi, A, Silvagni E., Chessa E., Bergossi F., D'Amico M. E., Furini F., Guerrini G., Cauli A., Scire C. A., Bertsias G., Govoni M., Piga M., and Bortoluzzi A.
Abstract: Objectives: Although neuropsychiatric involvement in SLE (NPSLE) is one of the most complex and troubling manifestations of the disease, validated outcome instruments to be used as sensitive endpoints in controlled clinical trials are lacking. We performed a systematic literature review (SLR) to identify outcome measurement instruments and domains used to assess NPSLE. Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were used. Articles available in English (1967-2020), listed in PubMed, Embase, PsycINFO, Cochrane Library and the EULAR outcome measures library were screened. All domains and outcome measurement instruments were characterized according to the OMERACT Filter 2.1, considering core areas (manifestations/abnormalities, life impact, death/lifespan, societal/resource use) and contextual factors. Results: Of 3392 abstracts evaluated, 83 studies were included in the SLR (15 974 patients, females 89.9%). Eligible studies included domains and instruments pertinent to all core areas defined by the OMERACT, except for 'societal/resource use'. The most common core areas were 'manifestations/abnormalities', covering 10 domains pertinent to laboratory and instrumental markers, indexes and neuropsychiatric dimension (cognitive, neurologic and psychiatric field), and 'life impact', covering 7 domains related to physical function (from both the perspective of the patient and the physician), pain and quality of life. Conclusion: Our study revealed great heterogeneity in the instruments derived from populations with NPSLE and none of these had high-quality evidence. This supports the need to develop and further validate a core domain set and outcome measurement instruments to promote clinical research in this field, enhancing comparability across studies.
Published: 2022

11. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology

Author: De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., Sebastiani G. D., De Angelis, R., Ferri, C., Giuggioli, D., Bajocchi, G., Dagna, L., Bellando-Randone, S., Zanframundo, G., Foti, Roberta, Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Lepri, G., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A. M., Ciano, G., Beretta, Carlo Luigi, Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Cipolletta, Eleonora, Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Di Vico, C., Gigante, A., Pellagrino, G., Pigatto, E., Lazzaroni, M. -G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, Maria Assunta, Carrara, Giancarlo, Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, Enrica Maria, Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Guiducci, S., Doria, A., Salvarani, C., Iannone, F., Matucci-Cerinic, M., Giorgio, A., Alessia, B., Francesca, C., Renato, C., Dall'Ara, F., Angelo, D. C., Marica, D., Gianluca, S., Rossella, T., Foti R., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Cipolletta E., Zanetti A., Carrara G., Fusaro E., and Sebastiani G. D.
Abstract: Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort. Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets. Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud's phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1-16.5) than lcSSc (2 years, IQR 0-7), and dcSSc (1 year, IQR 0-3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001). Conclusion The ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.
Published: 2023

12. Diagnostic accuracy of a velcro sound detector (VECTOR) for interstitial lung disease in rheumatoid arthritis patients: the InSPIRAtE validation study (INterStitial pneumonia in rheumatoid ArThritis with an electronic device)

Author: Manfredi, A., Cassone, G., Cerri, S., Venerito, V., Fedele, A. L., Trevisani, M., Furini, F., Addimanda, O., Pancaldi, F., Della Casa, G., D’Amico, R., Vicini, R., Sandri, G., Torricelli, P., Celentano, I., Bortoluzzi, A., Malavolta, N., Meliconi, R., Iannone, F., Gremese, E., Luppi, F., Salvarani, C., Sebastiani, M., and on behalf of GISEA (Gruppo Italiano Studio Early Arthritis)
Published: 2019
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13. Quality of life and therapeutic management of axial spondyloarthritis patients in Italy: A 12-month prospective observational study

Author: D Angelo, S, Malavolta, N, Scambi, C, Salvarani, C, Caso, F, Tirri, E, Ramonda, R, Quarta, L, Erre, G, Riva, M, Buono, R, Furini, F, Grembiale, R, Lomater, C, Cantini, F, Maio, T, Chimenti, M, Scrivo, R, Salaffi, F, Caporali, R, Volpe, P, Gualberti, G, Marando, F, Marchesoni, A, D Angelo S., Malavolta N., Scambi C., Salvarani C., Caso F., Tirri E., Ramonda R., Quarta L., Erre G. L., Riva M., Buono R., Furini F., Grembiale R. D., Lomater C., Cantini F., Maio T., Chimenti M. S., Scrivo R., Salaffi F., Caporali R. F., Volpe P., Gualberti G., Marando F., Marchesoni A., D Angelo, S, Malavolta, N, Scambi, C, Salvarani, C, Caso, F, Tirri, E, Ramonda, R, Quarta, L, Erre, G, Riva, M, Buono, R, Furini, F, Grembiale, R, Lomater, C, Cantini, F, Maio, T, Chimenti, M, Scrivo, R, Salaffi, F, Caporali, R, Volpe, P, Gualberti, G, Marando, F, Marchesoni, A, D Angelo S., Malavolta N., Scambi C., Salvarani C., Caso F., Tirri E., Ramonda R., Quarta L., Erre G. L., Riva M., Buono R., Furini F., Grembiale R. D., Lomater C., Cantini F., Maio T., Chimenti M. S., Scrivo R., Salaffi F., Caporali R. F., Volpe P., Gualberti G., Marando F., and Marchesoni A.
Abstract: Objective To evaluate the health-related quality of life (HRQoL), disease activity, treatment adherence, and work ability in the real-world setting in patients with axial spondyloarthritis (axSpA). Methods QUASAR was a prospective 12-month, observational study involving 23 rheumatology centres across Italy, including adult patients with axSpA according to the Assessment of SpondyloArthritis International Society (ASAS) criteria. Patients were followed at baseline, 3, 6, and 12 months for disease activity and health-related QoL (HRQoL), treatment adherence and work ability. Regression analysis was used to assess the association between treatment and outcome variables. Results 413 (80.7%) out of axSpA 512 patients were diagnosed with ankylosing spondylitis (AS) and 99 (19.3%) with non-radiographic axSpA (nr-AxSpA). Nr-AxSpA and AS patients had similar baseline disease activity and HRQoL. Biologic disease-modifying anti-rheumatic drugs (bDMARDs) were the most frequent medication (n=426, 83.2%). Over the 1-year follow-up, disease activity measures (joint pain and swelling, CRP, global assessment, BASDAI, ASDAS), HRQoL and work ability significantly improved, while few differences emerged between nr-AxSpA and AS patients. Treatment satisfaction and adherence questionnaires improved over the 12 months. Patients treated with bDMARDs showed improved outcomes for disease activity measures and HRQoL variables, greater benefit observed in patients with AS. Conclusion We found clinical and HRQoL improvement over 1 year in a large, real-world population of nr-AxSpA and AS patients treated with bDMARDs or conventional synthetic DMARDs.
Published: 2021

14. Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of scleroderma spectrum

Author: Ferri, C, Giuggioli, D, Guiducci, S, Lumetti, F, Bajocchi, G, Magnani, L, Codullo, V, Ariani, A, Girelli, F, Riccieri, V, Pellegrino, G, Bosello, S, Foti, R, Visalli, E, Amato, G, Benenati, A, Cuomo, G, Iannone, F, Cacciapaglia, F, De Angelis, R, Ingegnoli, F, Talotta, R, Campochiaro, C, Dagna, L, De Luca, G, Bellando-Randone, S, Spinella, A, Murdaca, G, Romeo, N, De Santis, M, Generali, E, Barsotti, S, Della Rossa, A, Cavazzana, I, Dall'Ara, F, Lazzaroni, M, Cozzi, F, Doria, A, Pigatto, E, Zanatta, E, Ciano, G, Beretta, L, Abignano, G, D'Angelo, S, Mennillo, G, Bagnato, G, Calabrese, F, Caminiti, M, Pagano Mariano, G, Battaglia, E, Lubrano, E, Zanframundo, G, Iuliano, A, Furini, F, Zanetti, A, Carrara, G, Rumi, F, Scirè, C, Matucci-Cerinic, M, Ferri C, Giuggioli D, Guiducci S, Lumetti F, Bajocchi G, Magnani L, Codullo V, Ariani A, Girelli F, Riccieri V, Pellegrino G, Bosello S, Foti R, Visalli E, Amato G, Benenati A, Cuomo G, Iannone F, Cacciapaglia F, De Angelis R, Ingegnoli F, Talotta R, Campochiaro C, Dagna L, De Luca G, Bellando-Randone S, Spinella A, Murdaca G, Romeo N, De Santis M, Generali E, Barsotti S, Della Rossa A, Cavazzana I, Dall'Ara F, Lazzaroni MG, Cozzi F, Doria A, Pigatto E, Zanatta E, Ciano G, Beretta L, Abignano G, D'Angelo S, Mennillo G, Bagnato G, Calabrese F, Caminiti M, Pagano Mariano G, Battaglia E, Lubrano E, Zanframundo G, Iuliano A, Furini F, Zanetti A, Carrara G, Rumi F, Scirè CA, Matucci-Cerinic M, Ferri, C, Giuggioli, D, Guiducci, S, Lumetti, F, Bajocchi, G, Magnani, L, Codullo, V, Ariani, A, Girelli, F, Riccieri, V, Pellegrino, G, Bosello, S, Foti, R, Visalli, E, Amato, G, Benenati, A, Cuomo, G, Iannone, F, Cacciapaglia, F, De Angelis, R, Ingegnoli, F, Talotta, R, Campochiaro, C, Dagna, L, De Luca, G, Bellando-Randone, S, Spinella, A, Murdaca, G, Romeo, N, De Santis, M, Generali, E, Barsotti, S, Della Rossa, A, Cavazzana, I, Dall'Ara, F, Lazzaroni, M, Cozzi, F, Doria, A, Pigatto, E, Zanatta, E, Ciano, G, Beretta, L, Abignano, G, D'Angelo, S, Mennillo, G, Bagnato, G, Calabrese, F, Caminiti, M, Pagano Mariano, G, Battaglia, E, Lubrano, E, Zanframundo, G, Iuliano, A, Furini, F, Zanetti, A, Carrara, G, Rumi, F, Scirè, C, Matucci-Cerinic, M, Ferri C, Giuggioli D, Guiducci S, Lumetti F, Bajocchi G, Magnani L, Codullo V, Ariani A, Girelli F, Riccieri V, Pellegrino G, Bosello S, Foti R, Visalli E, Amato G, Benenati A, Cuomo G, Iannone F, Cacciapaglia F, De Angelis R, Ingegnoli F, Talotta R, Campochiaro C, Dagna L, De Luca G, Bellando-Randone S, Spinella A, Murdaca G, Romeo N, De Santis M, Generali E, Barsotti S, Della Rossa A, Cavazzana I, Dall'Ara F, Lazzaroni MG, Cozzi F, Doria A, Pigatto E, Zanatta E, Ciano G, Beretta L, Abignano G, D'Angelo S, Mennillo G, Bagnato G, Calabrese F, Caminiti M, Pagano Mariano G, Battaglia E, Lubrano E, Zanframundo G, Iuliano A, Furini F, Zanetti A, Carrara G, Rumi F, Scirè CA, and Matucci-Cerinic M
Abstract: Objectives: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). Methods: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. Results: Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features. Conclusions: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.
Published: 2020

15. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author: de Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., de Santis, M., Murdaca, G., Abignano, G., Romeo, N., Rossa, A. D., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., de Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., de Luca, G., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Dall'Ara, F., Lazzaroni, M. G., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Risa, A. M., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Ferri, C., Bosello S. (ORCID:0000-0002-4837-447X), Sebastiani G. D., de Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., de Santis, M., Murdaca, G., Abignano, G., Romeo, N., Rossa, A. D., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., de Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., de Luca, G., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Dall'Ara, F., Lazzaroni, M. G., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Risa, A. M., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Ferri, C., Bosello S. (ORCID:0000-0002-4837-447X), and Sebastiani G. D.
Abstract: Objective. There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics. Methods. A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared. Results. The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs. Conclusion. Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.
Published: 2022

16. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Author: Ferri, C., De Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Lazzaroni, M. G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Bosello S. (ORCID:0000-0002-4837-447X), Sebastiani G. D., Ferri, C., De Angelis, R., Giuggioli, D., Bajocchi, G., Dagna, L., Zanframundo, G., Foti, R., Cacciapaglia, F., Cuomo, G., Ariani, A., Rosato, E., Guiducci, S., Girelli, F., Riccieri, V., Zanatta, E., Bosello, Silvia Laura, Cavazzana, I., Ingegnoli, F., De Santis, M., Murdaca, G., Abignano, G., Romeo, N., Della Rossa, A., Caminiti, M., Iuliano, A., Ciano, G., Beretta, L., Bagnato, G., Lubrano, E., De Andres, I., Giollo, A., Saracco, M., Agnes, C., Lumetti, F., Spinella, A., Magnani, L., Campochiaro, C., De Luca, G., Codullo, V., Visalli, E., Masini, F., Gigante, A., Bellando-Randone, S., Pellegrino, G., Pigatto, E., Lazzaroni, M. G., Franceschini, F., Generali, E., Mennillo, G., Barsotti, S., Mariano, G. P., Calabrese, F., Furini, F., Vultaggio, L., Parisi, S., Peroni, C. L., Rozza, D., Zanetti, A., Carrara, G., Landolfi, G., Scire, C. A., Bianchi, G., Fusaro, E., Sebastiani, Gian Domenico, Govoni, M., D'Angelo, S., Cozzi, F., Doria, A., Iannone, F., Salvarani, C., Matucci-Cerinic, M., Bosello S. (ORCID:0000-0002-4837-447X), and Sebastiani G. D.
Abstract: Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature. Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 ± 26.9 yrs.; mean disease duration 8.9 ± 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas. Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches. Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.
Published: 2022

17. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study

Author: Bettiol, A., Urban, M. L., Dagna, L., Cottin, V., Franceschini, F., Del Giacco, S., Schiavon, F., Neumann, T., Lopalco, G., Novikov, P., Baldini, C., Lombardi, C., Berti, A., Alberici, F., Folci, M., Negrini, S., Sinico, R. A., Quartuccio, L., Lunardi, C., Parronchi, P., Moosig, F., Espigol-Frigole, G., Schroeder, J., Kernder, A. L., Monti, S., Silvagni, E., Crimi, C., Cinetto, F., Fraticelli, P., Roccatello, D., Vacca, A., Mohammad, A. J., Hellmich, B., Samson, M., Bargagli, E., Cohen Tervaert, J. W., Ribi, C., Fiori, D., Bello, F., Fagni, F., Moroni, L., Ramirez, G. A., Nasser, M., Marvisi, C., Toniati, P., Firinu, D., Padoan, R., Egan, A., Seeliger, B., Iannone, F., Salvarani, C., Jayne, D., Prisco, D., Vaglio, A., Emmi, G., Ahmad, K., Beccalli, M., Bonnotte, B., Bortolotti, R., Cariddi, A., Caminati, M., Cid, M. C., Deidda, M., Delvino, P., Scala, G. D., Felicetti, M., Ferro, F., Furini, F., Gelain, E., Ghirelli, G., Holle, J., Losappio, L. M., Mahr, A., Malandrino, D., Marhhold, J., Mattioli, I., Moi, L., Moiseev, S., Muratore, F., Nolasco, S., Olivieri, B., Palermo, A., Regola, F., Sander, O., Scarpa, R., Sciascia, S., Silvestri, E., Susca, N., Terrier, B., Treppo, E., Trezzi, B., Uzzo, M., Vitiello, G., Yacyshyn, E., RS: MHeNs - R3 - Neuroscience, Faculteit FHML Centraal, Bettiol, A, Urban, M, Dagna, L, Cottin, V, Franceschini, F, Del Giacco, S, Schiavon, F, Neumann, T, Lopalco, G, Novikov, P, Baldini, C, Lombardi, C, Berti, A, Alberici, F, Folci, M, Negrini, S, Sinico, R, Quartuccio, L, Lunardi, C, Parronchi, P, Moosig, F, Espígol-Frigolé, G, Schroeder, J, Kernder, A, Monti, S, Silvagni, E, Crimi, C, Cinetto, F, Fraticelli, P, Roccatello, D, Vacca, A, Mohammad, A, Hellmich, B, Samson, M, Bargagli, E, Cohen Tervaert, J, Ribi, C, Fiori, D, Bello, F, Fagni, F, Moroni, L, Ramirez, G, Nasser, M, Marvisi, C, Toniati, P, Firinu, D, Padoan, R, Egan, A, Seeliger, B, Iannone, F, Salvarani, C, Jayne, D, Prisco, D, Vaglio, A, Emmi, G, Jayne, David [0000-0002-1712-0637], and Apollo - University of Cambridge Repository
Subjects: Male, Epidemiology, Birmingham Vasculitis Activity Score, law.invention, Glucocorticoid, Randomized controlled trial, Prednisone, law, Eosinophilic, Monoclonal, Immunology and Allergy, Humanized, PLACEBO, Middle Aged, egpa mepolizumab, Treatment Outcome, SAFETY, Female, ANCA-associated Vasculitis, Biologicals, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss), Glucocorticoids, Granulomatosis with polyangiitis, ANCA-associated Vasculiti, medicine.drug, Keywords: ANCA-associated Vasculitis, Adult, medicine.medical_specialty, Immunology, Antibodies, Monoclonal, Humanized, Antibodies, Drug Administration Schedule, Eosinophilia, Granulomatosis with Polyangiitis, Humans, Retrospective Studies, Rheumatology, Internal medicine, medicine, Adverse effect, Asthma, business.industry, medicine.disease, Biological, EGPA, European EGPA Study Group, business, FOLLOW-UP, Mepolizumab
Abstract: OBJECTIVE: Mepolizumab proved to be an efficacious treatment for eosinophilic granulomatosis with polyangiitis (EGPA) at a dose of 300 mg every 4 weeks in the randomized, controlled MIRRA trial. In a few recently reported studies, successful real-life experiences with the approved dose for treating severe eosinophilic asthma (100 mg every 4 weeks) were observed. We undertook this study to assess the effectiveness and safety of mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks in a large European EGPA cohort. METHODS: We included all patients with EGPA treated with mepolizumab at the recruiting centers in 2015-2020. Treatment response was evaluated from 3 months to 24 months after initiation of mepolizumab. Complete response to treatment was defined as no disease activity (Birmingham Vasculitis Activity Score [BVAS] = 0) and a prednisolone or prednisone dose (or equivalent) of ��4 mg/day. Respiratory outcomes included asthma and ear, nose, and throat (ENT) exacerbations. RESULTS: Two hundred three patients, of whom 191 received a stable dose of mepolizumab (158 received 100 mg every 4 weeks and 33 received 300 mg every 4 weeks) were included. Twenty-five patients (12.3%) had a complete response to treatment at 3 months. Complete response rates increased to 30.4% and 35.7% at 12 months and 24 months, respectively, and rates were comparable between mepolizumab 100 mg every 4 weeks and 300 mg every 4 weeks. Mepolizumab led to a significant reduction in BVAS score, prednisone dose, and eosinophil counts from 3 months to 24 months, with no significant differences observed between 100 mg every 4 weeks and 300 mg every 4 weeks. Eighty-two patients (40.4%) experienced asthma exacerbations (57 of 158 [36%] who received 100 mg every 4 weeks; 17 of 33 [52%] who received 300 mg every 4 weeks), and 31 patients (15.3%) experienced ENT exacerbations. Forty-four patients (21.7%) experienced adverse events (AEs), most of which were nonserious AEs (38 of 44). CONCLUSION: Mepolizumab at both 100 mg every 4 weeks and 300 mg every 4 weeks is effective for the treatment of EGPA. The 2 doses should be compared in the setting of a controlled trial.
Published: 2022
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18. Factors associated with fertility abnormalities in women with systemic lupus erythematosus: a systematic review and meta-analysis

Author: Giambalvo, S, Garaffoni, C, Silvagni, E, Furini, F, Rizzo, R, Govoni, M, and Bortoluzzi, A
Subjects: Adult, Immunology, Ambientale, Primary Ovarian Insufficiency, Systemic Lupus Erythematosus, Cross-Sectional Studies, Fertility, Systematic review, LS8_2, Immunology and Allergy, Humans, Lupus Erythematosus, Systemic, Female, Cyclophosphamide
Abstract: Fertility is thought to be not affected in women with systemic lupus erythematosus (SLE), however disease-related factors, psychosocial effects of chronic disease, as well as medications exposure might impair gonadal function.This systematic literature review (SLR) aimed to explore clinical, hormonal, serological and treatment factors associated with fertility outcomes in women of childbearing age with SLE.This SLR was conducted following the Preferred Reporting Items for systematic reviews and Meta-analysis (PRISMA) statement. All articles available in English (1972 - 30th April 2021) in Pubmed, EMBASE, Scopus and Cochrane Library were screened. Studies selection and data collection were performed by two independent reviewers. All data were extracted using a standardized template. The risk of bias of the included studies was assessed using the NIH risk-of-bias tool.Of 789 abstracts evaluated, we included in this review 46 studies, of which 1 SLR, 16 cross-sectional studies, 18 cohort studies, 10 observational studies and 1 case-series, with data pertaining to 4704 patients (mean age 31.5 ± 3.7 years, disease duration 83.27 ± 38.3 months). Definitions of premature ovarian failure (POF) adopted in the studies varied in terms of the number of months of amenorrhea considered and the age of onset of amenorrhea. Clinical factors associated with the development of POF were older age at the time of initiation of therapy, and older age at the onset of SLE disease. Cyclophosphamide exposure (CYC) and its cumulative dose influenced gonadal function in SLE women, leading to amenorrhoea and POF, as reported in 19 studies. Mycophenolate, azathioprine, calcineurin inhibitors and steroids associated with a lower risk of POF compared to CYC. POF was less frequent in patients co-treated with CYC and gonadotropin-releasing hormone analogues (GnRH-a) compared with patients not receiving GnRH-a (risk ratio 0.28, 95%-CI [0.14; 0.55]). 11 studies evaluated the impact of damage accrual and disease activity on ovarian reserve with conflicting evidence. Finally, 18 studies investigated exposure to hormonal and serological factors and, among others, neither anti-Müllerian Hormone nor anti-corpus luteum antibodies were associated with POF.The strongest evidence regarding management factors associated with fertility in SLE women of childbearing age remains the treatment with CYC, as well as its cumulative dosage. Hormonal and serological factors appeared not to impact fertility outcomes, but they might be used as a surrogate of fertility, especially during the treatment with disease-specific drugs.
Published: 2021

19. Diagnostic accuracy of a velcro sound detector (VECTOR) for interstitial lung disease in rheumatoid arthritis patients: The InSPIRAtE validation study (INterStitial pneumonia in rheumatoid ArThritis with an electronic device)

Author: Manfredi, A, Cassone, G, Cerri, S, Venerito, V, Fedele, A, Trevisani, M, Furini, F, Addimanda, O, Pancaldi, F, Della Casa, G, D'Amico, R, Vicini, R, Sandri, G, Torricelli, P, Celentano, I, Bortoluzzi, A, Malavolta, N, Meliconi, R, Iannone, F, Gremese, E, Luppi, F, Salvarani, C, Sebastiani, M, Manfredi A., Cassone G., Cerri S., Venerito V., Fedele A. L., Trevisani M., Furini F., Addimanda O., Pancaldi F., Della Casa G., D'Amico R., Vicini R., Sandri G., Torricelli P., Celentano I., Bortoluzzi A., Malavolta N., Meliconi R., Iannone F., Gremese E., Luppi F., Salvarani C., Sebastiani M., Manfredi, A, Cassone, G, Cerri, S, Venerito, V, Fedele, A, Trevisani, M, Furini, F, Addimanda, O, Pancaldi, F, Della Casa, G, D'Amico, R, Vicini, R, Sandri, G, Torricelli, P, Celentano, I, Bortoluzzi, A, Malavolta, N, Meliconi, R, Iannone, F, Gremese, E, Luppi, F, Salvarani, C, Sebastiani, M, Manfredi A., Cassone G., Cerri S., Venerito V., Fedele A. L., Trevisani M., Furini F., Addimanda O., Pancaldi F., Della Casa G., D'Amico R., Vicini R., Sandri G., Torricelli P., Celentano I., Bortoluzzi A., Malavolta N., Meliconi R., Iannone F., Gremese E., Luppi F., Salvarani C., and Sebastiani M.
Abstract: Background: Interstitial lung disease (ILD) is a severe systemic manifestation of rheumatoid arthritis (RA). High-resolution computed tomography (HRCT) represents the gold standard for the diagnosis of ILD, but its routine use for screening programs is not advisable because of both high cost and X-ray exposure. Velcro crackles at lung auscultation occur very early in the course of interstitial pneumonia, and their detection is an indication for HRCT. Recently, we developed an algorithm (VECTOR) to detect the presence of Velcro crackles in pulmonary sounds and showed good results in a small sample of RA patients. The aim of the present investigation was to validate the diagnostic accuracy of VECTOR in a larger population of RA patients, compared with that of the reference standard of HRCT, from a multicentre study. Methods: To avoid X-ray exposure, we enrolled 137 consecutive RA patients who had recently undergone HRCT. Lung sounds of all patients were recorded in 4 pulmonary fields bilaterally with a commercial electronic stethoscope (ES); subsequently, all HRCT images were blindly evaluated by a radiologist, and audio data were analysed by means of VECTOR. Results: Fifty-nine of 137 patients showed ILD (43.1%). VECTOR correctly classified 115/137 patients, showing a diagnostic accuracy of 83.9% and a sensitivity and specificity of 93.2 and 76.9%, respectively. Conclusions: VECTOR may represent the first validated tool for the screening of RA patients who are suspected for ILD and who should be directed to HRCT for the diagnosis. Moreover, early identification of RA-ILD could contribute to the design of prospective studies aimed at elucidating unclear aspects of the disease.
Published: 2019

20. The role of the multidisciplinary evaluation of interstitial lung diseases: Systematic literature review of the current evidence and future perspectives

Author: Furini, F, Carnevale, A, Casoni, G, Guerrini, G, Cavagna, L, Govoni, M, Scire, C, Furini F., Carnevale A., Casoni G. L., Guerrini G., Cavagna L., Govoni M., Scire C. A., Furini, F, Carnevale, A, Casoni, G, Guerrini, G, Cavagna, L, Govoni, M, Scire, C, Furini F., Carnevale A., Casoni G. L., Guerrini G., Cavagna L., Govoni M., and Scire C. A.
Abstract: The opportunity of a multidisciplinary evaluation for the diagnosis of interstitial pneumonias highlighted a major change in the diagnostic approach to diffuse lung disease. The new American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Latin American Thoracic Society guidelines for the diagnosis of idiopathic pulmonary fibrosis have reinforced this assumption and have underlined that the exclusion of connective tissue disease related lung involvement is mandatory, with obvious clinical and therapeutic impact. The multidisciplinary team discussion consists in amoment of interaction among the radiologist, pathologist and pulmonologist, also including the rheumatologist when considered necessary, to improve diagnostic agreement and optimize the definition of those cases in which pulmonary involvement may represent the first or prominent manifestation of an autoimmune systemic disease. Moreover, the proposal of classification criteria for interstitial lung disease with autoimmune features (IPAF) represents an effort to define lung involvement in clinically undefined autoimmune conditions. The complexity of autoimmune diseases, and in particular the lack of classification criteria defined for pathologies such as anti-synthetase syndrome, makes the involvement of the rheumatologist essential for the correct interpretation of the autoimmune element and for the application of classification criteria, that could replace clinical pictures initially interpreted as IPAF in defined autoimmune disease, minimizing the risk of misdiagnosis. The aim of this review was to evaluate the available evidence about the efficiency and efficacy of different multidisciplinary team approaches, in order to standardize the professional figures and the core set procedures that should be necessary for a correct approach in diagnosing patients with interstitial lung disease.
Published: 2019

21. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author: Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., and Cavagna L.
Abstract: Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
Published: 2019

22. Nailfold capillaroscopy characteristics of antisynthetase syndrome and possible clinical associations: Results of a multicenter international study

Author: Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani M., Triantafyllias K., Manfredi A., Gonzalez-Gay M. A., Palmou-Fontana N., Cassone G., Drott U., Delbruck C., Rojas-Serrano J., Bertolazzi C., Nuno L., Giannini M., Iannone F., Vicente E. F., Castaneda S., Selva-O'Callaghan A., Araguas E. T., Emmi G., Iuliano A., Bauhammer J., Miehle N., Parisi S., Cavagna L., Codullo V., Montecucco C., Lopez-Longo F. J., Martinez-Barrio J., Nieto-Gonzalez J. C., Vichi S., Confalonieri M., Tomietto P., Bergner R., Sulli A., Bonella F., Furini F., Scire C. A., Bortoluzzi A., Specker C., Barsotti S., Neri R., Mosca M., Caproni M., Weinmann-Menke J., Schwarting A., Smith V., Cutolo M., Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani M., Triantafyllias K., Manfredi A., Gonzalez-Gay M. A., Palmou-Fontana N., Cassone G., Drott U., Delbruck C., Rojas-Serrano J., Bertolazzi C., Nuno L., Giannini M., Iannone F., Vicente E. F., Castaneda S., Selva-O'Callaghan A., Araguas E. T., Emmi G., Iuliano A., Bauhammer J., Miehle N., Parisi S., Cavagna L., Codullo V., Montecucco C., Lopez-Longo F. J., Martinez-Barrio J., Nieto-Gonzalez J. C., Vichi S., Confalonieri M., Tomietto P., Bergner R., Sulli A., Bonella F., Furini F., Scire C. A., Bortoluzzi A., Specker C., Barsotti S., Neri R., Mosca M., Caproni M., Weinmann-Menke J., Schwarting A., Smith V., and Cutolo M.
Abstract: Objective. To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease. Methods. We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non-anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody-negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures. Results. NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP. Conclusion. NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.
Published: 2019

23. Risk of acute arterial and venous thromboembolic events in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

Author: Bettiol, A., Sinico, R. A., Schiavon, F., Monti, S., Bozzolo, E. P., Franceschini, F., Govoni, M., Lunardi, C., Guida, G., Lopalco, G., Paolazzi, G., Vacca, A., Gregorini, G., Leccese, P., Piga, M., Conti, F., Fraticelli, P., Quartuccio, L., Alberici, F., Salvarani, C., Bettio, S., Negrini, S., Selmi, C., Sciascia, S., Moroni, G., Colla, L., Manno, C., Urban, M. L., Vannacci, A., Pozzi, M. R., Fabbrini, P., Polti, S., Felicetti, M., Marchi, M. R., Padoan, R., Delvino, P., Caporali, R., Montecucco, C., Dagna, L., Cariddi, A., Toniati, P., Tamanini, S., Furini, F., Bortoluzzi, A., Tinazzi, E., Delfino, L., Badiu, I., Rolla, G., Venerito, V., Iannone, F., Berti, A., Bortolotti, R., Racanelli, V., Jeannin, G., Padula, A., Cauli, A., Priori, R., Gabrielli, A., Bond, M., Tedesco, M., Pazzola, G., Tomietto, P., Pellecchio, M., Marvisi, C., Maritati, F., Palmisano, A., Dejaco, C., Willeit, J., Kiechl, S., Olivotto, I., Willeit, P., Prisco, D., Vaglio, A., Emmi, G., Bargagli, E., Becatti, M., Beccalli, M., Bello, F., Bozzao, F., Canti, V., Cassia, M. A., Cassone, G., Catanoso, M., Chieco-Bianchi, F., Clari, R., Coladonato, L., De Santis, M., Di Scala, G., Fagni, F., Fenaroli, P., Fiorillo, C., Floris, A., Fornaro, M., Galli, E., Generali, E., Giliberti, M., Lascaro, N., Leccese, I., Mattioli, I., Olivieri, B., Osti, N., Peyronel, F., Radin, M., Righetti, G., Salvati, S., Silvestri, E., Susca, N., Tamburini, C., Taurisano, G., Trezzi, B., Trivioli, G., Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, and Emmi, G
Subjects: Pulmonary and Respiratory Medicine, Burden of disease, Humans, Churg-Strauss Syndrome, Granulomatosis with Polyangiitis, Venous Thromboembolism, Venous Thrombosis, Churg-strauss syndrome, Criminology, NO, 03 medical and health sciences, 0302 clinical medicine, Medicine, 030212 general & internal medicine, Vascular inflammation, business.industry, Conflict of interest, Cytoplasmic antibody, medicine.disease, 030228 respiratory system, Wegener granulomatosis, arterial and venous thromboembolic events, Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome), Organ involvement, business, Production team
Abstract: Eosinophilic Granulomatosis with Polyangiitis (EGPA, Churg-Strauss syndrome) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) characterised by respiratory manifestations and systemic organ involvement [1]. Particularly, cardiac manifestations occur in 40–60% of patients, representing the leading cause of mortality [2]. Recent reports suggest that venous thromboembolic events might also represent a consistent burden of disease [3, 4], as already known for the other AAVs [5–7], possibly due to eosinophil-mediated vascular inflammation [5]. Nevertheless, the occurrence of arterial and venous thrombotic events (AVTE) has never been systematically explored in EGPA. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Alessandra Bettiol Conflict of interest: Renato Alberto Sinico Conflict of interest: Franco Schiavon Conflict of interest: Sara Monti Conflict of interest: Enrica Paola Bozzolo Conflict of interest: Franco Franceschini Conflict of interest: Marcello Govoni Conflict of interest: Claudio Lunardi Conflict of interest: Giuseppe Guida Conflict of interest: Giuseppe Lopalco Conflict of interest: Giuseppe Paolazzi Conflict of interest: Angelo Vacca Conflict of interest: Gina Gregorini Conflict of interest: Pietro Leccese Conflict of interest: Matteo Piga Conflict of interest: Fabrizio Conti Conflict of interest: Paolo Fraticelli Conflict of interest: Luca Quartuccio Conflict of interest: Federico Alberici Conflict of interest: Carlo Salvarani Conflict of interest: Silvano Bettio Conflict of interest: Simone Negrini Conflict of interest: Carlo Selmi Conflict of interest: Savino Sciascia Conflict of interest: Gabriella Moroni Conflict of interest: Loredana Colla Conflict of interest: Carlo Manno Conflict of interest: Maria Letizia Urban Conflict of interest: Alfredo Vannacci Conflict of interest: Maria Rosa Pozzi Conflict of interest: Paolo Fabbrini Conflict of interest: Stefano Polti Conflict of interest: Mara Felicetti Conflict of interest: Maria Rita Marchi Conflict of interest: Roberto Padoan Conflict of interest: Paolo Delvino Conflict of interest: Roberto Caporali Conflict of interest: Carlomaurizio Montecucco Conflict of interest: Lorenzo Dagna Conflict of interest: Adriana Cariddi Conflict of interest: Paola Toniati Conflict of interest: Dr. Tamanini reports other from Glaxo Smith Kline, outside the submitted work. Conflict of interest: Federica Furini Conflict of interest: Alessandra Bortoluzzi Conflict of interest: Elisa Tinazzi Conflict of interest: Lorenzo Delfino Conflict of interest: Iuliana Badiu Conflict of interest: Giovanni Rolla Conflict of interest: Vincenzo Venerito Conflict of interest: Florenzo Iannone Conflict of interest: Alvise Berti Conflict of interest: Roberto Bortolotti Conflict of interest: Vito Racanelli Conflict of interest: Guido Jeannin Conflict of interest: Angela Padula Conflict of interest: Alberto Cauli Conflict of interest: Roberta Priori Conflict of interest: Armando Gabrielli Conflict of interest: Milena Bond Conflict of interest: Martina Tedesco Conflict of interest: Giulia Pazzola Conflict of interest: Paola Tomietto Conflict of interest: Marco Pellecchio Conflict of interest: Chiara Marvisi Conflict of interest: Federica Maritati Conflict of interest: Alessandra Palmisano Conflict of interest: Christian Dejaco Conflict of interest: Johann Willeit Conflict of interest: Stefan Kiechl Conflict of interest: Iacopo Olivotto Conflict of interest: Peter Willeit Conflict of interest: Domenico Prisco Conflict of interest: Augusto Vaglio Conflict of interest: Giacomo Emmi
Published: 2020

24. Radiotherapy in cancer and rheumathoid arthritis patients: cancer treatment or control of articular flares? We can achieve both

Author: Fiorica, F., Ciancio, G., Giuliani, J., Bonetti, A., Berretta, M., Guarneri, C., Giorgi, C., Furini, F., Guerra, V., and Govoni, M.
Subjects: Male, Radiotherapy, Cancer, Rheumatoid arthritis, NO, Arthritis, Rheumatoid, Antirheumatic Agents, Neoplasms, Radiotherapy, Rheumatoid arthritis, Cancer, Humans, Female, Aged, Retrospective Studies
Abstract: The study was aimed to investigate the role of radiotherapy (RT) as a risk factor for reactivation or worsening of symptoms in patients affected by rheumatoid arthritis (RA) PATIENTS AND METHODS: This is a single-center retrospective observational study on RA patients who developed cancer requiring RT during the course of the disease. The control group consisted of RA patients with cancer who did not undergo RT. In both groups, the disease activity was evaluated at baseline and at 6 and 12 months through the DAS28 index. A relapse was defined as an increase of20% in DAS28. A radiotherapist evaluated total and daily doses and timing of radiation. Acute and late toxicity was defined as events occurring within 90 days from the start and more than 90 days after the completion of RT, respectively.Seventy-two RA patients (38F/34M; mean age: 70±9 years; mean disease duration: 13±9 years), 29 (40.2%) of whom received radiotherapy (mean age 72.9±9 years), were enrolled. The most frequent malignancies were breast (27.2%), thyroid (9.8%), and skin (7%). Between radio-treated and non-radio-treated patients, no significant differences in RA reactivation (6/29 vs. 17/43; p=0.12) or mean exacerbation time (6.7 ± 4.9 months compared to 6.4 ± 4.1 months; p=0.78) were found. Overall, RT was well tolerated with low rates of both acute and late toxicity.In RA patients, RT was well tolerated and not associated with an increased risk of articular flares. Properly designed prospective clinical studies with a larger number of patients should be performed to confirm these data.
Published: 2021

25. Impact of disease duration and gender on the performance of 2015 ACR/EULAR classification criteria for gout. Cross-sectional results from an Italian multicentric study on the management of crystal-induced arthritis (ATTACk)

Author: Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Ceccarelli, F, Lucchetti, R, Bortoluzzi, A, Amadeo Cimmino, M, Di Matteo, A, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Furini, F, Zanetti, A, Carrara, G, Scire', C, Doria, A, Ramonda, R, Mariagrazia Lorenzin, Nicola Ughi, Alarico Ariani, Bernd Raffeiner, Fulvia Ceccarelli, Ramona Lucchetti, Alessandra Bortoluzzi, Marco Amadeo Cimmino, Andrea Di Matteo, Paola Frallonardo, Ariela Hoxha, Augusta Ortolan, Marta Favero, Simone Parisi, Federica Furini, Anna Zanetti, Greta Carrara, Carlo Alberto Scirè, Andrea Doria, Roberta Ramonda, Lorenzin, M, Ughi, N, Ariani, A, Raffeiner, B, Ceccarelli, F, Lucchetti, R, Bortoluzzi, A, Amadeo Cimmino, M, Di Matteo, A, Frallonardo, P, Hoxha, A, Ortolan, A, Favero, M, Parisi, S, Furini, F, Zanetti, A, Carrara, G, Scire', C, Doria, A, Ramonda, R, Mariagrazia Lorenzin, Nicola Ughi, Alarico Ariani, Bernd Raffeiner, Fulvia Ceccarelli, Ramona Lucchetti, Alessandra Bortoluzzi, Marco Amadeo Cimmino, Andrea Di Matteo, Paola Frallonardo, Ariela Hoxha, Augusta Ortolan, Marta Favero, Simone Parisi, Federica Furini, Anna Zanetti, Greta Carrara, Carlo Alberto Scirè, Andrea Doria, and Roberta Ramonda
Abstract: OBJECTIVES: We aimed to assess the performance of the 2015 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) gout classification criteria in an Italian cohort of patients with crystal-induced arthritis stratified by disease duration and gender in a real-life setting. METHODS: Consecutive patients referred to Rheumatology Units for suspected acute crystal-induced arthritis were enrolled in a multicentre cohort study by the Italian Society of Rheumatology which was designed to improve the management of crystal-induced arthritis (ATTACk). To test the performance of the criteria (sensitivity and specificity), the presence of monosodium urate (MSU) crystals in synovial fluid (SF) was used as gold standard. Subgroup analyses by gender and disease duration were performed. RESULTS: Two hundred and seventy-seven patients were enrolled. SF analysis was available in 137 (49%) patients. Complete SF analysis and ACR/EULAR scores were obtained in 44% of patients. MSU crystals were found in 66% of patients. The sensitivity and the specificity of all criteria sets were 78% (95%CI, 67–86) and 98% (95%CI, 87–100), respectively; only clinical criteria yielded 70% (95%CI, 59–80) sensitivity and 93% (95%CI, 80-98) specificity, respectively. In early-stage disease (<2 years), the sensitivity dropped to 58% (95%CI, 39-75), while the specificity was 100% (95%CI, 85–100). CONCLUSIONS: The ACR/EULAR criteria showed good performance in patients presenting with acute arthritis; changes were observed when a subset of criteria were used, especially in early-stage disease.
Published: 2021

26. Risk of acute arterial and venous thromboembolic events in Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)

Author: Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, Emmi, Giacomo, Bettiol, A, Sinico, R, Schiavon, F, Monti, S, Bozzolo, E, Franceschini, F, Govoni, M, Lunardi, C, Guida, G, Lopalco, G, Paolazzi, G, Vacca, A, Gregorini, G, Leccese, P, Piga, M, Conti, F, Fraticelli, P, Quartuccio, L, Alberici, F, Salvarani, C, Bettio, S, Negrini, S, Selmi, C, Sciascia, S, Moroni, G, Colla, L, Manno, C, Urban, M, Vannacci, A, Pozzi, M, Fabbrini, P, Polti, S, Felicetti, M, Marchi, M, Padoan, R, Delvino, P, Caporali, R, Montecucco, C, Dagna, L, Cariddi, A, Toniati, P, Tamanini, S, Furini, F, Bortoluzzi, A, Tinazzi, E, Delfino, L, Badiu, I, Rolla, G, Venerito, V, Iannone, F, Berti, A, Bortolotti, R, Racanelli, V, Jeannin, G, Padula, A, Cauli, A, Priori, R, Gabrielli, A, Bond, M, Tedesco, M, Pazzola, G, Tomietto, P, Pellecchio, M, Marvisi, C, Maritati, F, Palmisano, A, Dejaco, C, Willeit, J, Kiechl, S, Olivotto, I, Willeit, P, Prisco, D, Vaglio, A, Emmi, G, Bettiol, Alessandra, Sinico, Renato Alberto, Schiavon, Franco, Monti, Sara, Bozzolo, Enrica Paola, Franceschini, Franco, Govoni, Marcello, Lunardi, Claudio, Guida, Giuseppe, Lopalco, Giuseppe, Paolazzi, Giuseppe, Vacca, Angelo, Gregorini, Gina, Leccese, Pietro, Piga, Matteo, Conti, Fabrizio, Fraticelli, Paolo, Quartuccio, Luca, Alberici, Federico, Salvarani, Carlo, Bettio, Silvano, Negrini, Simone, Selmi, Carlo, Sciascia, Savino, Moroni, Gabriella, Colla, Loredana, Manno, Carlo, Urban, Maria Letizia, Vannacci, Alfredo, Pozzi, Maria Rosa, Fabbrini, Paolo, Polti, Stefano, Felicetti, Mara, Marchi, Maria Rita, Padoan, Roberto, Delvino, Paolo, Caporali, Roberto, Montecucco, Carlomaurizio, Dagna, Lorenzo, Cariddi, Adriana, Toniati, Paola, Tamanini, Silvia, Furini, Federica, Bortoluzzi, Alessandra, Tinazzi, Elisa, Delfino, Lorenzo, Badiu, Iuliana, Rolla, Giovanni, Venerito, Vincenzo, Iannone, Florenzo, Berti, Alvise, Bortolotti, Roberto, Racanelli, Vito, Jeannin, Guido, Padula, Angela, Cauli, Alberto, Priori, Roberta, Gabrielli, Armando, Bond, Milena, Tedesco, Martina, Pazzola, Giulia, Tomietto, Paola, Pellecchio, Marco, Marvisi, Chiara, Maritati, Federica, Palmisano, Alessandra, Dejaco, Christian, Willeit, Johann, Kiechl, Stefan, Olivotto, Iacopo, Willeit, Peter, Prisco, Domenico, Vaglio, Augusto, and Emmi, Giacomo
Published: 2021

27. POS0716 FACTORS ASSOCIATED WITH FERTILITY OUTCOMES IN WOMEN WITH SYSTEMIC LUPUS ERYTHEMATOSUS: A SYSTEMATIC REVIEW

Author: Giambalvo, S., primary, Garaffoni, C., additional, Silvagni, E., additional, Furini, F., additional, Govoni, M., additional, and Bortoluzzi, A., additional
Published: 2021
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28. One year in review 2018: Novelties in the treatment of rheumatoid arthritis

Author: Bortoluzzi, A, Furini, F, Generali, E, Silvagni, E, Luciano, N, Scire, C, Bortoluzzi A., Furini F., Generali E., Silvagni E., Luciano N., Scire C. A., Bortoluzzi, A, Furini, F, Generali, E, Silvagni, E, Luciano, N, Scire, C, Bortoluzzi A., Furini F., Generali E., Silvagni E., Luciano N., and Scire C. A.
Abstract: The current approach to treatment of rheumatoid arthritis (RA) includes early and aggressive intervention aiming to reach early and persistent low disease activity and remission. New drugs have improved the therapeutic armamentarium of rheumatologists, providing new options for patients. Beyond these innovations, new evidence has improved the safety of therapies and provided tools for the optimisation of long-term management of RA. This paper reviews the most relevant studies published over the last year in the field of treatment of RA.
Published: 2018

29. Osteoarthritis and its management - Epidemiology, nutritional aspects and environmental factors

Author: Bortoluzzi, A, Furini, F, Scire, C, Bortoluzzi A., Furini F., Scire C. A., Bortoluzzi, A, Furini, F, Scire, C, Bortoluzzi A., Furini F., and Scire C. A.
Abstract: Osteoarthritis (OA) is the most prevalent chronic rheumatic diseases worldwide, with a strong impact on individual and population health. OA is a clinically heterogeneous disease presenting with different clinical phenotypes recognising systemic and local risk factors. The pathogenesis is multifactorial including constitutive features of the joint, non-modifiable and modifiable risk factors. Epidemiological studies highlight the link between metabolic syndrome and OA and the effect of interplay between immunological and metabolic processes is getting increasing emphasis because of to the discovery that metabolic syndrome is implicated in OA pathogenesis and progression. In addition, recent findings suggest a potential role of dietary factors in susceptibility and progression of OA. In this review, we summarise the most robust evidence on epidemiology and classical risk factors OA, also exploring the most recent evidence on metabolic changes and Mediterranean diet for OA as a possible target to impact on the natural history of the disease.
Published: 2018

30. Racial differences in systemic sclerosis disease presentation: A European Scleroderma Trials and Research group study

Author: Jaeger, Veronika K, Tikly, Mohammed, Dong, Xu, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Mengtao, Li, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich, A, Randone, Sb, Bannert, B, Iannone, F, Maurer, B, Jordan, S, Dobrota, R, Becker, M, Mihai, C, Becvarare, R, Tomčík, M, Bielecka, Ok, Gindzienska-Sieskiewicz, E, Karaszewska, K, Cutolo, M, Pizzorni, C, Paolino, S, Sulli, A, Ruaro, B, Alessandri, E, Riccardi, A, Giacco, V, Messitini, V, Irace, R, Kedor, C, Casteleyn, V, Hilger, J, Hoeppner, J, Rednic, S, Szabo, I, Petcu, A, Avouac, J, Camelia, F, Desbas, C, Vlachoyiannopoulos, P, Montecucco, C, Caporali, R, Cavagna, L, Stork, J, Inanc, M, Joven, Be, Novak, S, Anic, F, Varju, C, Minier, T, Chizzolini, C, Allai, D, Kucharz, Ej, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Dolnicar, As, Coleiro, B, Gabrielli, A, Manfredi, L, Benfaremo, D, Ferrarini, A, Bancel, Df, Hij, A, Lansiaux, P, Lazzaroni, Mg, Hesselstrand, R, Wuttge, D, Andréasson, R, Martinovic, D, Bozic, I, Radic, M, Braun-Moscovici, Y, Monaco, Al, Furini, F, Hunzelmann, N, Moinzadeh, P, Pellerito, R, Caimmi, C, Bertoldo, E, Morovic-Vergles, J, Culo, Im, Pecher, Ac, Santamaria, Vo, Heitmann, S, Codagnone, M, Pflugfelder, J, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Hasler, P, Kretschmar, S, Kohm, M, Bajocchi, G, Salvador, Mj, Silva, Japd, Stamenkovic, B, Stankovic, A, Selmi, Cf, Santis, M, Ceribelli, A, Garzanova, L, Koneva, O, Starovoytova, M, Herrick, A, Puppo, F, Negrini, S, Murdaca, G, Engelhart, M, Szücs, G, Szamosi, S, de la Puente, C, Grande, Cs, Villanueva, Mjg, Midtvedt, Sø, Hoffmann-Vold, Am, Launay, D, Sobanski, V, Riccieri, V, Vasile, M, Ionescu, Rm, Opris, D, Sha, A, Woods, A, Gheorghiu, Am, Bojinca, M, Sunderkötter, C, Ehrchen, J, Ingegnoli, F, Mouthon, L, Dunogue, B, Chaigne, B, Legendre, P, Cantatore, Fp, Corrado, A, Ullman, S, Iversen, L, von Mühlen CA, Pozzi, Mr, Eyerich, K, Lauffer, F, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Vanthuyne, M, Frédéric, H, Alegre-Sancho, Jj, Aringer, M, Herrmann, K, Günther, C, Westhovens, R, Langhe, E, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Üprus, M, Otsa, K, Yavuz, S, Granel, B, Radominski, Sc, De, C, Müller, S, Azevedo, Vf, Mendoza, F, Busquets, J, Popa, S, Agachi, S, Zenone, T, Pileckyte, M, Stebbings, S, Mathieu, A, Vacca, A, Sampaio-Barros, Pd, Stamp, L, Solanki, K, Silva, C, Schollum, J, Barns-Graham, H, Veale, D, O'Rourke, M, Loyo, E, Tineo, C, Paulino, G, Mohamed, Waaa, Rosato, E, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, Cm, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Visalli, E, Benenati, A, Amato, G, Ancuta, C, Villiger, P, Adler, S, Fröhlich, J, Kayser, C, Eduardo, Al, Fathi, N, Alii, S, Ahmed, M, Hasaneen, S, Hakeem, Ee, de la PG, Lefebvre, P, Martin, Jjg, Sibilia, J, Chatelus, E, Gottenberg, Je, Chifflot, H, Litinsky, I, Galdo, Fd, Abignano, G, Eng, S, Seskute, G, Butrimiene, I, Rugiene, R, Karpec, D, Pascal, M, Kerzberg, E, Bianchi, W, Bianchi, Bv, Bianchi, Dv, Barcellos, Y, Castellví, I, Millan, M, Limonta, M, Rimar, D, Rosner, I, Slobodin, G, Couto, M, Spertini, F, Ribi, C, Buss, G, Marcoccia, A, Bondanini, F, Ciani, A, Kahl, S, Hsu, Vm, Martin, T, Poindron, V, Meghit, K, Moiseev, S, Novikov, P, Chung, L, Kolstad, K, Stark, M, Schmeiser, T, Thiele, A, Majewski, D, Zdrojewski, Z, Zaneta, S, Wierzba, K, Martínez-Barrio, J, López-Longo, Fj, Bernardino, V, Moraes-Fontes, Mf, Rodrigues, Ac, Riemekasten, G, Sommerlatte, S, Jendreck, S, Arnold, S, Levy, Y, Rezus, E, Cardoneanu, A, Burlui, Am, Pamuk, On, Puttini, Ps, Talotta, R, Bongiovanni, S, Poormoghim, H, Andalib, E, Almasi, S, Kötter, I, Krusche, M, Cuomo, G, Danzo, F, Masini, F, Gaches, F, Michaud, M, Cartos, F, Belloli, L, Casu, C, Sfikakis, P, Tektonidou, M, Furst, D, Feldman, Gr, Ramazan, Am, Nurmambet, E, Miroto, A, Suta, C, Andronache, I, Huizinga, Twj, de Vries-Bouwstra, J., Chizzolini, Carlo, Jaeger, Veronika K, Tikly, Mohammed, Xu, Dong, Siegert, Elise, Hachulla, Eric, Airò, Paolo, Valentini, Gabriele, Matucci Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia, Allanore, Yannick, Müller-Ladner, Ulf, Ananieva, Lidia P, Balbir-Gurman, Alexandra, Distler, Jörg H W, Czirják, Laszlo, Li, Mengtao, Henes, Jörg, Jimenez, Sergio A, Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P, Delgaldo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich A, University of Zurich, Cerinic, Marco Matucci, Walker Ulrich, A, Randone, Silvia Bellando, Bannert, Bettina, Iannone, Florenzoaa, Maurer, Brittaab, Jordan, Suzanaab, Dobrota, Rucsandraab, Becker, Mikeab, Mihai, Carinaa, Becvarare, Radima, Tomcik, Michala, Bielecka, Otylia Kowala, Gindzienska-Sieskiewicz, Ewaa, Karaszewska, Katarzynaa, Cutolo, Maurizioa, Pizzorni, Carmena, Paolino, Sabrinaae, Sulli, Albertoa, Ruaro, Barbara, Alessandri, Elisa, Riccardi, Antonella, Giacco, Veronica, Messitini, Valentina, Irace, Rosaria, Kedor, Claudia, Casteleyn, Vincent, Hilger, Julia, Hoeppner, Jakob, Rednic, Simona, Szabo, Iulia, Petcu, Ana, Avouac, Jérome, Camelia, Frantz, Desbas, Carole, Vlachoyiannopoulos, Panayioti, Montecucco, Carlo Maurizio, Caporali, Roberto, Cavagna, Lorenzo, Stork, Jiri, Inanc, Murat, Joven, Beatriz E., Novak, Srdan, Anic, Felina, Varju, Cecilia, Minier, Tunde, Allai, Daniela, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Dolnicar, Alenka Sipek, Coleiro, Bernard, Gabrielli, Armando, Manfredi, Lucia, Benfaremo, Devi, Ferrarini, Alessia, Bancel, Dominique Farge, Hij, Adrian, Lazzaroni, Maria Grazia, Hesselstrand, Roger, Wuttge, Dirk, Andréasson, Kristofer, Martinovic, Duska, Bozic, Ivona, Radic, Mislav, Braun-Moscovici, Yolanda, Monaco, Andrea Lo, Furini, Federica, Hunzelmann, Nicola, Moinzadeh, Pia, Pellerito, Raffaele, Caimmi, Cristian, Bertoldo, Eugenia, Morovic-Vergles, Jadranka, Culo, Ivana Melanie, Pecher, Ann-Christian, Santamaria, Vera Ortiz, Heitmann, Stefan, Codagnone, Medeleine, Pflugfelder, Johanne, Krasowska, Dorota, Michalska-Jakubus, Malgorzata, Seidel, Matthia, Hasler, Paul, Kretschmar, Samuel, Kohm, Michaela, Bajocchi, Gianluigi, Salvador, Maria João, Da Silva, JoséAntonio Pereira, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Ceribelli, Angela, Garzanova, Ludmila, Koneva, Olga, Starovoytova, Maya, Herrick, Ariane, Puppo, Francesco, Negrini, Simone, Murdaca, Giuseppe, Engelhart, Merete, Szücs, Gabriela, Szamosi, Szilvia, De La Puente, Carlo, Grande, Cristina Sobrino, Villanueva, Maria Jesus Garcia, Midtve, Øyvindbw, Hoffmann-Vold, Anna-Mariabw, Launay, Davidbx, Sobanski, Vincentbx, Riccieri, Valeriaby, Vasile, Massimilianoby, Stefantoni, Katia, Ionescu, Ruxandra Maria, Opris, Daniela, Sha, Ami, Woods, Adrianne, Gheorghiu, Ana Maria, Bojinca, Mihai, Sunderkötter, Cord, Ehrchen, Jan, Ingegnoli, Francesca, Mouthon, Luc, Dunogue, Bertrand, Chaigne, Benjamin, Legendre, Paul, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, Von Mühlen, Carlos Alberto, Pozzi, Maria Rosa, Eyerich, Kilian, Lauffer, Felix, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Vanthuyne, Marie, Frédéric, Houssiau, Alegre-Sancho, Juan Jose, Aringer, Martin, Herrmann, Kristine, Günther, Claudia, Westhovens, Rene, De Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Üprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastião Cezar, De Souza Müller, Carolina, Feijóazevedo, Valderílio, Mendoza, Fabian, Busquets, Joanna, Popa, Sergei, Agachi, Svetlana, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Jordan, Sarah, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D., Stamp, Lisa, Solanki, Kamal, Silva, Cherumi, Schollum, Joanne, Barns-Graham, Helen, Veale, Dougla, O'Rourke, Marie, Loyo, Esthela, Tineo, Carmen, Paulino, Glenny, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Visalli, Elisa, Benenati, Alessia, Amato, Giorgio, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Fröhlich, Johanne, Kayser, Cristiane, Eduardo, Andrade Lui, Fathi, Nihal, Alii, Safa, Ahmed, Marrow, Hasaneen, Samar, El Hakeem, Eman, De La Peña Lefebvre, Paloma García, Martin, Jorge Juan Gonzalez, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Del Galdo, Francesco, Abignano, Giuseppina, Eng, Sookho, Seskute, Goda, Butrimiene, Irena, Rugiene, Rita, Karpec, Diana, Pascal, Melanie, Kerzberg, Eduardo, Bianchi, Washington, Bianchi, Breno Valdetaro, Bianchi, Dante Valdetaro, Barcellos, Yeda, Castellví, Ivan, Millan, Milena, Limonta, Massimiliano, Rimar, Doron, Rosner, Itzhak, Slobodin, Gleb, Couto, Maura, Spertini, Françoi, Ribi, Camillo, Buss, Guillaume, Marcoccia, Antonella, Bondanini, Francesco, Ciani, Aldo, Kahl, Sarah, Hsu, Vivien M., Martin, Thierry, Poindron, Vincent, Meghit, Kilifa, Moiseev, Sergey, Novikov, Pavel, Chung, Lori, Kolstad, Kathleen, Stark, Marianna, Schmeiser, Tim, Thiele, Astrid, Majewski, Dominik, Zdrojewski, Zbigniew, Zaneta, Smolenska, Wierzba, Karol, Martínez-Barrio, Julia, López-Longo, Francisco Javier, Bernardino, Vera, Moraes-Fontes, Maria Francisca, Rodrigues, Ana Catarina, Riemekasten, Gabriela, Sommerlatte, Sabine, Jendreck, Sebastian, Arnold, Sabrina, Levy, Yair, Rezus, Elena, Cardoneanu, Anca, Burlui, Alexandra Maria, Pamuk, Omer Nuri, Puttini, Piercarlo Sarzi, Talotta, Rossella, Bongiovanni, Sara, Poormoghim, Hadi, Andalib, Elham, Almasi, Simin, Kötter, Ina, Krusche, Matrin, Cuomo, Giovanna, Danzo, Fiammetta, Masini, Francesco, Gaches, Franci, Michaud, Martin, Cartos, Florian, Belloli, Laura, Casu, Cinzia, Sfikakis, Petro, Tektonidou, Maria, Furst, Daniel, Feldman, Gary R., Ramazan, Ana-Maria, Nurmambet, Emel, Miroto, Amalia, Suta, Cristina, Andronache, Iulia, Huizinga, Tom W. J., De Vries-Bouwstra, Jeska, and Walker, Ulrich A.
Subjects: Male, Vital capacity, Organ manifestations, systemic sclerosis, Type I, race difference, Systemic scleroderma, Gastroenterology, Scleroderma, immunology, 0302 clinical medicine, Diffusing capacity, middle aged, pulmonary hypertension, Medicine, Pharmacology (medical), 030212 general & internal medicine, organ manifestations, races, skin and connective tissue diseases, Lung, race, pathophysiology, African Continental Ancestry Group, ddc:616, integumentary system, disease course, Hazard ratio, Races, 10051 Rheumatology Clinic and Institute of Physical Medicine, Pulmonary, Middle Aged, Blacks, cohort analysis, Autoantibodie, 3. Good health, Asians, female, priority journal, DNA Topoisomerases, Type I, Black, centromere, Cohort, Hypertension, organ manifestation, Systemic sclerosis, Female, systemic sclerosi, Human, Adult, Asian Continental Ancestry Group, medicine.medical_specialty, Hypertension, Pulmonary, European Continental Ancestry Group, Black People, 610 Medicine & health, complication, Caucasian, White People, Article, lung, 03 medical and health sciences, Black person, Rheumatology, Asian People, forced vital capacity, Internal medicine, geographic distribution, Humans, controlled study, human, DNA topoisomerase, Aged, Autoantibodies, 030203 arthritis & rheumatology, Scleroderma, Systemic, Asian, business.industry, Whites, Systemic, Odds ratio, medicine.disease, Pulmonary hypertension, major clinical study, mortality, clinical feature, business, DNA Topoisomerases, autoantibody
Abstract: Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.
Published: 2020

31. Systemic sclerosis progression INvestiGation (SPRING) Italian registry: Demographic and clinico-serological features of the scleroderma spectrum

Author: Ferri, C., Giuggioli, D., Guiducci, S., Lumetti, F., Bajocchi, G., Magnani, L., Codullo, V., Ariani, A., Girelli, F., Riccieri, V., Pellegrino, G., Bosello, Silvia Laura, Foti, Roberta, Visalli, E., Amato, G., Benenati, A., Cuomo, G., Iannone, F., Cacciapaglia, F., de Angelis, R., Ingegnoli, F., Talotta, R., Campochiaro, C., Dagna, L., de Luca, G., Bellando-Randone, S., Spinella, A., Murdaca, G., Romeo, N., de Santis, M., Generali, E., Barsotti, S., della Rossa, A., Cavazzana, I., Dall’Ara, F., Lazzaroni, M. G., Cozzi, F., Doria, A., Pigatto, E., Zanatta, E., Ciano, G., Beretta, Carlo Luigi, Abignano, G., D’Angelo, S., Mennillo, G. A., Bagnato, G., Calabrese, F., Caminiti, M., Pagano Mariano, G., Battaglia, E., Lubrano, E., Zanframundo, G., Iuliano, Angela, Furini, F., Zanetti, Maria Assunta, Carrara, Giancarlo, Rumi, Filippo, Scirè, C. A., Matucci-Cerinic, M., Bosello S. (ORCID:0000-0002-4837-447X), Foti R., Beretta L. (ORCID:0000-0001-9924-2066), Iuliano A., Zanetti A., Carrara G., Rumi F., Ferri, C., Giuggioli, D., Guiducci, S., Lumetti, F., Bajocchi, G., Magnani, L., Codullo, V., Ariani, A., Girelli, F., Riccieri, V., Pellegrino, G., Bosello, Silvia Laura, Foti, Roberta, Visalli, E., Amato, G., Benenati, A., Cuomo, G., Iannone, F., Cacciapaglia, F., de Angelis, R., Ingegnoli, F., Talotta, R., Campochiaro, C., Dagna, L., de Luca, G., Bellando-Randone, S., Spinella, A., Murdaca, G., Romeo, N., de Santis, M., Generali, E., Barsotti, S., della Rossa, A., Cavazzana, I., Dall’Ara, F., Lazzaroni, M. G., Cozzi, F., Doria, A., Pigatto, E., Zanatta, E., Ciano, G., Beretta, Carlo Luigi, Abignano, G., D’Angelo, S., Mennillo, G. A., Bagnato, G., Calabrese, F., Caminiti, M., Pagano Mariano, G., Battaglia, E., Lubrano, E., Zanframundo, G., Iuliano, Angela, Furini, F., Zanetti, Maria Assunta, Carrara, Giancarlo, Rumi, Filippo, Scirè, C. A., Matucci-Cerinic, M., Bosello S. (ORCID:0000-0002-4837-447X), Foti R., Beretta L. (ORCID:0000-0001-9924-2066), Iuliano A., Zanetti A., Carrara G., and Rumi F.
Abstract: inglese
Published: 2020

32. FRI0239 ANTI-NXP2 ANTIBODIES: CLINICAL AND SEROLOGICAL ASSOCIATIONS IN A MULTICENTRIC ITALIAN STUDY

Author: Fredi, M., primary, Cavazzana, I., additional, Ceribelli, A., additional, Lazzaroni, M. G., additional, Barsotti, S., additional, Benucci, M., additional, Cavagna, L., additional, De Stefano, L., additional, Doria, A., additional, Emmi, G., additional, Fornaro, M., additional, Furini, F., additional, Gerli, R., additional, Giudizi, M. G., additional, Govoni, M., additional, Ghirardello, A., additional, Iaccarino, L., additional, Iannone, F., additional, Infantino, M., additional, Mathieu, A., additional, Marasco, E., additional, Migliorini, P., additional, Palterer, B., additional, Parronchi, P., additional, Piga, M., additional, Pratesi, F., additional, Radice, A., additional, Selmi, C., additional, Riccieri, V., additional, Tampoia, M., additional, Zanframundo, G., additional, Tincani, A., additional, and Franceschini, F., additional
Published: 2020
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33. THU0150 INTERSTITIAL LUNG DISEASE RELATED TO RHEUMATOID ARTHRITIS. WHAT DO WE DON’T KNOW? THE LIRA STUDY (LUNG INVOLVEMENT IN RHEUMATOID ARTHRITIS).

Author: Sebastiani, M., primary, Vacchi, C., additional, Cassone, G., additional, Atzeni, F., additional, Biggioggero, M., additional, Carriero, A., additional, Erre, G. L., additional, Fedele, A. L., additional, Furini, F., additional, Tomietto, P., additional, Venerito, V., additional, Atienza-Mateo, B., additional, Della Casa, G., additional, Cerri, S., additional, Sandri, G., additional, Palermo, A., additional, Galli, E., additional, Pancaldi, F., additional, González-Gay, M. A., additional, Salvarani, C., additional, and Manfredi, A., additional
Published: 2020
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34. Conventional brain magnetic resonance imaging in the longitudinal evaluation of newly diagnosed systemic lupus erythematosus patients: a retrospective analysis from a single-centre cohort

Author: Silvagni, E, primary, Bortoluzzi, A, additional, Borrelli, M, additional, Padovan, M, additional, Furini, F, additional, and Govoni, M, additional
Published: 2020
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35. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author: Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, and Cavagna, L
Subjects: myositi
Abstract: Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.
Published: 2018

36. NEW PERSPECTIVES IN DIAGNOSIS OF INTERSTITIAL LUNG DISEASE RELATED TO RHEUMATOID ARTHRITIS. VALIDATION STUDY OF AN ELECTRONIC STETHOSCOPE AND AD HOC SOFTWARE FOR DETECTION OF PULMONARY CRACKLES

Author: Manfredi, A, Sebastiani, M, Cassone, G, Fedele, A, Venerito, V, Trevisani, M, Furini, F, Addimanda, O, Gremese, E, Iannone, F, DELLA CASA, G, Cerri, S, Sandri, G, Pancaldi, F, Luppi, F, Ferri, C, MANFREDI, Andreina Teresa, SEBASTIANI, Marco, Cassone, Giulia, Fedele, A. L., Venerito, V., Trevisani, M., Furini, F., Addimanda, O., Gremese, E., Iannone, F., DELLA CASA, GIOVANNI, CERRI, Stefania, SANDRI, Gilda, PANCALDI, Fabrizio, LUPPI, Fabrizio, FERRI, Clodoveo, Manfredi, A, Sebastiani, M, Cassone, G, Fedele, A, Venerito, V, Trevisani, M, Furini, F, Addimanda, O, Gremese, E, Iannone, F, DELLA CASA, G, Cerri, S, Sandri, G, Pancaldi, F, Luppi, F, Ferri, C, MANFREDI, Andreina Teresa, SEBASTIANI, Marco, Cassone, Giulia, Fedele, A. L., Venerito, V., Trevisani, M., Furini, F., Addimanda, O., Gremese, E., Iannone, F., DELLA CASA, GIOVANNI, CERRI, Stefania, SANDRI, Gilda, PANCALDI, Fabrizio, LUPPI, Fabrizio, and FERRI, Clodoveo
Abstract: Background: Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by synovial joint swelling and tenderness, secondary to the immune-system dysfunction, often complicated by extra-articular manifestations. Among them, lung involvement is very frequent and interstitial lung disease (ILD) represents one of the deleterious complications of RA with impact on both therapeutic approach and overall prognosis. Nevertheless, diagnosis of ILD often remains missing or delayed. Objectives: To preliminarily evaluate the predictive value of pulmonary sound recorded by an electronic stethoscope (ES) and elaborated by an ad hoc software in identification of RA-ILD diagnosed by mean of high resolution computed tomography (HRCT) in a multicenter study. Methods: RA patients who underwent HRCT in the last 12 months were enrolled. They were all auscultated with the ES (Littmann 3200TM 3M, USA), bilaterally, at dorsal level, in at least 3 pulmonary fields (medium and basal). All tracks recorded were analyzed by a suitably developed software capable of recognizing pathological crackles in lung sounds. Results were compared with radiologic findings detected in a blind manner by an expert radiologist. Results: One hundred and six RA patients were enrolled (M/F: 1/2.5, mean age 68.7±10.3); among them 45 (42.5%) showed ILD at HRCT. Three patients were excluded because of a low quality of the sound recorded. The algorithm showed a sensitivity and specificity of 72.1% and 84.4%, respectively and a positive/negative predictive value of 69.1% and 86.3%, respectively. Conclusions: Despite preliminary, these data suggest an important role of ES in clinical practice for an early diagnosis of ILD in RA patients and a significant reduction of inappropriate prescription of HRCT. Since very different types of ILD can occur in course of RA, with different radiologic features and localization, proper development of the measurement setup (ES and ad hoc software for the detection of PC)
Published: 2017

37. Serum Jo-1 Autoantibody and Isolated Arthritis in the Antisynthetase Syndrome: Review of the Literature and Report of the Experience of AENEAS Collaborative Group

Author: Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Sifuentes Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Pina Murcia, T, La Corte, R, Furini, F, Foschi, V, Bachiller Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bogliolo, L, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Salaffi, F, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Sifuentes Giraldo W. A., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Pina Murcia T., La Corte R., Furini F., Foschi V., Bachiller Corral J., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bogliolo L., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Salaffi F., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Sifuentes Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Pina Murcia, T, La Corte, R, Furini, F, Foschi, V, Bachiller Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bogliolo, L, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Salaffi, F, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Sifuentes Giraldo W. A., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Pina Murcia T., La Corte R., Furini F., Foschi V., Bachiller Corral J., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bogliolo L., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Salaffi F., Montecucco C., and Gonzalez-Gay M. A.
Abstract: Anti-Jo-1 is the most frequently detectable antibody in the antisynthetase syndrome (ASSD), an autoimmune disease characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). Recently, we organized an international collaborative group called American and European NEtwork of Antisynthetase Syndrome (AENEAS) for the study of this rare and fascinating disease. The group collected and published one of the largest series of ASSD patients ever described and with one of the longer follow-up ever reported. The number of participating centers is steadily increasing, as well as the available cohort. In the first paper, we showed that arthritis, myositis, and ILD may be frequently the only feature at disease onset, raising problems to reach a correct diagnosis of this syndrome. Nevertheless, we first observed that the ex novo appearance of further manifestations is common during the follow-up, strengthening the importance of a correct diagnosis. In our cohort, the 24 % of the 243 patients up to now collected had isolated arthritis as a presenting feature. These patients represent the most intriguing group in terms of differential diagnosis and clinical time course. Furthermore, data on this aspect are scanty, the reason that lead us to evaluate these aspects in our cohort of patients, reviewing also available literature. In fact, the most relevant aspect is that ASSD is rarely suspected in this setting of patients, in particular in case of poliarticular involvement, positive rheumatoid factor (RF), or anti-cyclic citrullinated peptide antibodies (ACPA) or evidence of joint erosions at plain radiographs. These findings were not rare in our cohort, and they have been also described in other series. Furthermore, manifestations such as Raynaud’s phenomenon, mechanic’s hands, and fever that may lead to the suspect of ASSD are observed only in a third of cases. If we consider the high rate of clinical picture progression in these patients, we fee
Published: 2017

38. Clinical follow-up predictors of disease pattern change in anti-Jo1 positive anti-synthetase syndrome: Results from a multicenter, international and retrospective study

Author: Bartoloni, E, Gonzalez-Gay, M, Scire, C, Castaneda, S, Gerli, R, Lopez-Longo, F, Martinez-Barrio, J, Govoni, M, Furini, F, Pina, T, Iannone, F, Giannini, M, Nuno, L, Quartuccio, L, Ortego-Centeno, N, Alunno, A, Specker, C, Montecucco, C, Triantafyllias, K, Balduzzi, S, Sifuentes-Giraldo, W, Paolazzi, G, Bravi, E, Schwarting, A, Pellerito, R, Russo, A, Selmi, C, Saketkoo, L, Fusaro, E, Parisi, S, Pipitone, N, Franceschini, F, Cavazzana, I, Neri, R, Barsotti, S, Codullo, V, Cavagna, L, Bartoloni E., Gonzalez-Gay M. A., Scire CA., Castaneda S., Gerli R., Lopez-Longo F. J., Martinez-Barrio J., Govoni M., Furini F., Pina T., Iannone F., Giannini M., Nuno L., Quartuccio L., Ortego-Centeno N., Alunno A., Specker C., Montecucco C., Triantafyllias K., Balduzzi S., Sifuentes-Giraldo W. A., Paolazzi G., Bravi E., Schwarting A., Pellerito R., Russo A., Selmi C., Saketkoo L. -A., Fusaro E., Parisi S., Pipitone N., Franceschini F., Cavazzana I., Neri R., Barsotti S., Codullo V., Cavagna L., Bartoloni, E, Gonzalez-Gay, M, Scire, C, Castaneda, S, Gerli, R, Lopez-Longo, F, Martinez-Barrio, J, Govoni, M, Furini, F, Pina, T, Iannone, F, Giannini, M, Nuno, L, Quartuccio, L, Ortego-Centeno, N, Alunno, A, Specker, C, Montecucco, C, Triantafyllias, K, Balduzzi, S, Sifuentes-Giraldo, W, Paolazzi, G, Bravi, E, Schwarting, A, Pellerito, R, Russo, A, Selmi, C, Saketkoo, L, Fusaro, E, Parisi, S, Pipitone, N, Franceschini, F, Cavazzana, I, Neri, R, Barsotti, S, Codullo, V, Cavagna, L, Bartoloni E., Gonzalez-Gay M. A., Scire CA., Castaneda S., Gerli R., Lopez-Longo F. J., Martinez-Barrio J., Govoni M., Furini F., Pina T., Iannone F., Giannini M., Nuno L., Quartuccio L., Ortego-Centeno N., Alunno A., Specker C., Montecucco C., Triantafyllias K., Balduzzi S., Sifuentes-Giraldo W. A., Paolazzi G., Bravi E., Schwarting A., Pellerito R., Russo A., Selmi C., Saketkoo L. -A., Fusaro E., Parisi S., Pipitone N., Franceschini F., Cavazzana I., Neri R., Barsotti S., Codullo V., and Cavagna L.
Abstract: Objective Arthritis, myositis and interstitial lung disease (ILD) constitute the classic clinical triad of anti-synthetase syndrome (ASSD). These patients experience other accompanying features, such as Raynaud's phenomenon, fever or mechanic's hands. Most ASSD patients develop the complete triad during the follow-up. In the present study we aimed to determine whether the subsequent appearance of accompanying features may suggest the development of triad findings lacking at the onset in anti-Jo1 positive ASSD patients. Methods Anti-Jo1 positive patients presenting with incomplete ASSD (no > 2 classic triad features) were assessed. Clinical characteristics and clusters of disease manifestations were retrospectively collected and analyzed in a large international multicenter cohort of ASSD patients. Results 165 patients (123 women) with incomplete ASSD were identified. Ninety-five patients (57.5%) developed new classic triad manifestations after 15 months median (IQR 9–51) and 40 (24%) developed new accompanying features after 19 months median (IQR 6–56) from disease onset. During the follow-up, the ex-novo occurrence of triad features was observed in 32 out of 40 patients (80%) with new accompanying findings and in 63 out of 125 patients (50.5%) without new accompanying findings (p = 0.002). In patients with at least one new accompanying feature the odds ratio for the occurrence of new triad manifestations was 3.94 with respect to patients not developing ex-novo accompanying findings (95% CI 1.68–9.21, p = 0.002). Conclusion Anti-Jo1 ASSD patients with incomplete forms at disease onset are at high risk for the subsequent occurrence of lacking classic triad findings. Although all ASSD patients should be carefully assessed for the occurrence of new triad features, a closer follow-up should be considered in the subgroup of patients developing ex novo accompanying findings. These patients, indeed, have near four-fold increased risk f
Published: 2017

39. SNP (1513A > C AND 489C > T) OF P2X7 RECEPTOR IN SYSTEMIC LUPUS ERYTHEMATOSUS WITH SEROSITIS

Author: Furini, F, Bortoluzzi, A, Giuliani, Al, Di Virgilio, F, and Govoni, M
Subjects: Socio-culturale
Published: 2019

40. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author: Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel
Abstract: Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.
Published: 2019

41. NEW PERSPECTIVES IN DIAGNOSIS OF INTERSTITIAL LUNG DISEASE RELATED TO RHEUMATOID ARTHRITIS. VALIDATION STUDY OF AN ELECTRONIC STETHOSCOPE AND AD HOC SOFTWARE FOR DETECTION OF PULMONARY CRACKLES

Author: MANFREDI, Andreina Teresa, SEBASTIANI, Marco, Cassone, Giulia, Fedele, A. L., Venerito, V., Trevisani, M., Furini, F., Addimanda, O., Gremese, E., Iannone, F., DELLA CASA, GIOVANNI, CERRI, Stefania, SANDRI, Gilda, PANCALDI, Fabrizio, LUPPI, Fabrizio, FERRI, Clodoveo, Manfredi, A, Sebastiani, M, Cassone, G, Fedele, A, Venerito, V, Trevisani, M, Furini, F, Addimanda, O, Gremese, E, Iannone, F, DELLA CASA, G, Cerri, S, Sandri, G, Pancaldi, F, Luppi, F, and Ferri, C
Subjects: interstitial lung disease, Rheumatoid arthritis, Rheumatoid arthriti
Abstract: Background: Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by synovial joint swelling and tenderness, secondary to the immune-system dysfunction, often complicated by extra-articular manifestations. Among them, lung involvement is very frequent and interstitial lung disease (ILD) represents one of the deleterious complications of RA with impact on both therapeutic approach and overall prognosis. Nevertheless, diagnosis of ILD often remains missing or delayed. Objectives: To preliminarily evaluate the predictive value of pulmonary sound recorded by an electronic stethoscope (ES) and elaborated by an ad hoc software in identification of RA-ILD diagnosed by mean of high resolution computed tomography (HRCT) in a multicenter study. Methods: RA patients who underwent HRCT in the last 12 months were enrolled. They were all auscultated with the ES (Littmann 3200TM 3M, USA), bilaterally, at dorsal level, in at least 3 pulmonary fields (medium and basal). All tracks recorded were analyzed by a suitably developed software capable of recognizing pathological crackles in lung sounds. Results were compared with radiologic findings detected in a blind manner by an expert radiologist. Results: One hundred and six RA patients were enrolled (M/F: 1/2.5, mean age 68.7±10.3); among them 45 (42.5%) showed ILD at HRCT. Three patients were excluded because of a low quality of the sound recorded. The algorithm showed a sensitivity and specificity of 72.1% and 84.4%, respectively and a positive/negative predictive value of 69.1% and 86.3%, respectively. Conclusions: Despite preliminary, these data suggest an important role of ES in clinical practice for an early diagnosis of ILD in RA patients and a significant reduction of inappropriate prescription of HRCT. Since very different types of ILD can occur in course of RA, with different radiologic features and localization, proper development of the measurement setup (ES and ad hoc software for the detection of PC) could further increase its predictive value, in particular to avoid incorrect records and misdiagnosis. The routinely employment of ES and proper software, combined to clinical findings (cough, dyspnea) and respiratory lung function, could increase our ability to early identify ILD in RA patients.
Published: 2017

42. Development and First Validation of a Disease Activity Score for Gout

Author: Scire, C, Carrara, G, Viroli, C, Cimmino, M, Taylor, W, Manara, M, Govoni, M, Salaffi, F, Punzi, L, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Scire C. A., Carrara G., Viroli C., Cimmino M. A., Taylor W. J., Manara M., Govoni M., Salaffi F., Punzi L., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., Veneto V., Scire, C, Carrara, G, Viroli, C, Cimmino, M, Taylor, W, Manara, M, Govoni, M, Salaffi, F, Punzi, L, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Scire C. A., Carrara G., Viroli C., Cimmino M. A., Taylor W. J., Manara M., Govoni M., Salaffi F., Punzi L., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., and Veneto V.
Abstract: Objective: To develop a new composite disease activity score for gout and provide its first validation. Methods: Disease activity has been defined as the ongoing presence of urate deposits that lead to acute arthritis and joint damage. Every measure for each Outcome Measures in Rheumatology core domain was considered. A 3-step approach (factor analysis, linear discriminant analysis, and linear regression) was applied to derive the Gout Activity Score (GAS). Decision to change treatment or 6-month flare count were used as the surrogate criteria of high disease activity. Baseline and 12-month followup data of 446 patients included in the Kick-Off of the Italian Network for Gout cohort were used. Construct- and criterion-related validity were tested. External validation on an independent sample is reported. Results: Factor analysis identified 5 factors: patient-reported outcomes, joint examination, flares, tophi, and serum uric acid (sUA). Discriminant function analysis resulted in a correct classification of 79%. Linear regression analysis identified a first candidate GAS including 12-month flare count, sUA, visual analog scale (VAS) of pain, VAS global activity assessment, swollen and tender joint counts, and a cumulative measure of tophi. Alternative scores were also developed. The developed GAS demonstrated a good correlation with functional disability (criterion validity) and discrimination between patient- and physician-reported measures of active disease (construct validity). The results were reproduced in the external sample. Conclusion: This study developed and validated a composite measure of disease activity in gout. Further testing is required to confirm its generalizability, responsiveness, and usefulness in assisting with clinical decisions.
Published: 2016

43. Clinical spectrum time course in anti jo-1 positive antisynthetase syndrome: Results from an international retrospective multicenter study

Author: Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Giraldo W. A. S., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Murcia T. P., La Corte R., Furini F., Foschi V., Corral J. B., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Montecucco C., Gonzalez-Gay M. A., Rosenthal K., Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Murcia, T, La Corte, R, Furini, F, Foschi, V, Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Montecucco, C, Gonzalez-Gay, M, Rosenthal, K, AENEAS (American, European NEtwork of Antisynthetase Syndrome) collaborative group, [Cavagna,L, Caporali,L, Montecucco,C] Division of Rheumatology, University and IRCCS Policlinico S. Matteo Foudation, Pavia, Italy. [Nuño,L] Servicio de Reumatología, Hospital Universitario La Paz, Madrid, Spain. [Scire,CA] Epidemiology Unit, Italian Society for Rheumatology, Milano, Italy. [Govoni ,M, Furini,F, La Corte,R, Foschi,V] UOC Reumatologia, Azienda Ospedaliero Universitaria S. Anna, University of Ferrara, Ferrara, Italy. [Lopez Longo.FJ, Martínez-Barrio,J, Hinojosa,M] Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain. [Franceschini,F, Airó,P, Cavazzana,I] Rheumatology Unit, University and AO Spedali Civili, Brescia, Italy. [Neri,R, Barsotti,S] Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy. [Castañeda,S] Department of Rheumatology, Hospital Universitario de la Princesa, IIS Princesa, Madrid, Spain.[Sifuentes Giraldo,WA, Bachiller Corral,AJ] Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain. [Iannone,F] Interdisciplinary Department of Medicine (DIM), Rheumatology Unit, University of Bari, Bari, Italy. [Fusaro,E, Parisi,S] Department of Rheumatology, Città Della Salute e della Scienza, Torino, Italy. [Paolazzi,G, Barausse,G, Bortolotti,R] Rheumatology Unit, Santa Chiara Hospital, Trento, Italy. [Pellerito,R, Vitetta,R, Russo,A] Division of Rheumatology, Mauriziano Hospital, Turin, Italy. [Schwarting,A, Menke,J] Department of Internal Medicine, Rheumatology and Clinical Immunology, University Hospital Johannes-Gutenberg, Mainz, Germany. [Saketkoo,LA] Tulane University Lung Center Tulane/UMC Scleroderma and Sarcoidosis Patient Care and Research Center, New Orleans, LA, USA. [Ortego-Centeno,N] Systemic Autoimmune Diseases Unit, Hospital Clínico San Cecilio, Granada, Spain. [Quartuccio,L] Santa Maria della Misericordia Hospital, Udine, Italy. [Bartoloni,E] Clinic of Rheumatology, Department of Medical and Biological Sciences. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy. [Specker,C] Department for Rheumatology and Clinical Immunology, St. Josef Krankenhaus, University Clinic, Essen, Germany. [Pina Murcia,T, and González-Gay,MA] Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. [Triantafyllias,K] ACURA Rheumatology Center, Bad Kreuznach, Germany. [Bajocchi,G] Rheumatology Unit, Department of Internal Medicine, S. Maria Hospital—IRCCS, Reggio Emilia, Italy. [Bravi,E] Rheumatology Unit, Ospedale Guglielmo da Saliceto, Piacenza, Italy. [Selmi,C] Division of Rheumatology and Clinical Immunology, Humanitas Research Hospital, Rozzano, Milano, Italy.
Subjects: Male, Pathology, Neurology, Anti Jo-1, Analytical, Diagnostic and Therapeutic Techniques and Equipment::Investigative Techniques::Epidemiologic Methods::Epidemiologic Study Characteristics as Topic::Epidemiologic Studies::Case-Control Studies::Retrospective Studies [Medical Subject Headings], Medizin, Arthritis, Antisynthetase syndrome, Organisms::Eukaryota::Animals::Chordata::Vertebrates::Mammals::Primates::Haplorhini::Catarrhini::Hominidae::Humans [Medical Subject Headings], Antinuclear, Masculino, Myositis, Persons::Persons::Age Groups::Adult::Aged [Medical Subject Headings], Medicine (all), Interstitial lung disease, Femenino, General Medicine, Middle Aged, Diseases::Musculoskeletal Diseases::Muscular Diseases::Myositis [Medical Subject Headings], Humanos, Anticuerpos antinucleares, Antibodies, Antinuclear, Diseases::Musculoskeletal Diseases::Joint Diseases::Arthritis [Medical Subject Headings], Female, Chemicals and Drugs::Amino Acids, Peptides, and Proteins::Proteins::Blood Proteins::Immunoproteins::Immunoglobulins::Antibodies::Autoantibodies::Antibodies, Antinuclear [Medical Subject Headings], Adult, medicine.medical_specialty, Check Tags::Male [Medical Subject Headings], Antibodies, NO, Estudios retrospectivos, Internal medicine, medicine, Humans, Risk factor, Aged, Retrospective Studies, Persons::Persons::Age Groups::Adult [Medical Subject Headings], Artritis, business.industry, Retrospective cohort study, Persons::Persons::Age Groups::Adult::Middle Aged [Medical Subject Headings], Anti Jo-1, Antisynthetase Syndrome, medicine.disease, Dermatology, Rheumatology, Check Tags::Female [Medical Subject Headings], Miositis, antisynthetase syndrome, business
Abstract: Anti Jo-1 antibodies are the main markers of the antisynthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined. The aim of this international, multicenter, retrospective study was to characterize the clinical time course of anti Jo-1 positive ASSD in a large cohort of patients. Included patients should be anti Jo-1 positive and with at least 1 feature between arthritis, myositis, and ILD. We evaluated the differences between complete and incomplete forms, timing of clinical picture appearance and analyzed factors predicting the appearance of further manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males and 167 females) with a median follow-up of 80 months. At the onset, complete ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently only 1 of the classic triad findings (110 cases), in particular, isolated arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28 cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5 patients had an isolated arthritis, only 5 an isolated myositis, and 15 an isolated ILD. During the follow-up, 108 patients with incomplete forms developed further manifestations. Single main feature onset was the main risk factor for the ex novo appearance of further manifestation. ILD was the prevalent ex novo manifestation (74 cases). In conclusion, ASSD is a condition that should be carefully considered in all patients presenting with arthritis, myositis, and ILD, even when isolated. The ex novo appearance of further manifestations in patients with incomplete forms is common, thus indicating the need for an adequate clinical and instrumental follow-up. Furthermore, the study clearly suggested that in ASSD multidisciplinary approach involving Rheumatology, Neurology, Pneumology, and Internal Medicine specialists is mandatory. CA extern
Published: 2015

44. Observational study on the QUality of life of Italian Axial SpondyloARthritis patients (QUASAR): baseline data

Author: D Angelo, S., Gilio, M., D Attino, R. M., Gualberti, G., Merolla, R., Di Luzio Paparatti, U., Malavolta, N., Corvaglia, S., Marchetta, A., Scambi, C., Romeo, N., Pettiti, G., Salvarani, C., Catanoso, M. G., Scarpa, R., Costa, L., Ramonda, R., Frallonardo, P., Muratore, M., Quarta, L., Passiu, G., Erre, G. L., Lubrano, D., Tirri, E., Govoni, M., Furini, F., Russo, R., Buono, R., Pozzi, M. R., Riva, M., Grembiale, R. D., Caterina Bruno, Gibertini, P., Marchesoni, A., D'Angelo, Salvatore, Gilio, Michele, D'Attino, Rita M, Gualberti, Giuliana, Merolla, Rocco, di Luzio Paparatti, Umberto, Malavolta, Nazzarena, Corvaglia, Stefania, Marchetta, Antonio, Scambi, Cinzia, Romeo, Nicoletta, Pettiti, Giorgio, Salvarani, Carlo, Catanoso, Maria Grazia, Scarpa, Raffaele, Costa, Luisa, Ramonda, Roberta, Frallonardo, Paola, Muratore, Maurizio, Quarta, Laura, Passiu, Giuseppe, Erre, Gian Luca, Lubrano, Daniele, Tirri, Enrico, Govoni, Marcello, Furini, Federica, Russo, Romualdo, Buono, Rosario, Pozzi, Maria Rosa, Riva, Marta, Grembiale, Rosa Daniela, Bruno, Caterina, Gibertini, Patrizia, and Marchesoni, Antonio
Subjects: Ankylosing, Adult, Male, ankylosing spondylitis, non-radiographic axial spondyloarthritis, quality of life, epidemiology, Italy, Anti-Inflammatory Agents, Socio-culturale, Young Adult, ankylosing spondylitis, Spondylarthritis, Humans, Spondylitis, Ankylosing, Prospective Studies, Spondylarthriti, Anti-Inflammatory Agents, Non-Steroidal, Antirheumatic Agent, Middle Aged, Prospective Studie, Italy, Antirheumatic Agents, Quality of Life, non-radiographic axial spondyloarthritis, epidemiology, Female, Non-Steroidal, Spondylitis, Human
Abstract: To describe the baseline characteristics of the patients enrolled in the QUality of life in patients with Axial SpondyloARthritis (QUASAR) study in terms of quality of life (QoL), disease activity, therapy adherence, and work ability in a real-world setting.QUASAR is an Italian multicentre, prospective 12-month observational study, including consecutive adult patients classified as axial spondyloarthritis (axSpA) according to the Assessment of SpondyloArthritis international Society criteria for axSpA.Of 512 patients enrolled in 23 rheumatology centres, 80.7% had ankylosing spondylitis (AS) and 19.3% had non-radiographic axSpA (nr-axSpA). Mean ages were 34.1±13.3 years at axSpA symptoms onset and 39.5±13.0 years at diagnosis. Of the patients, 51.4% presented with ≥1 extra articular manifestation (EAM); the most common were psoriasis (17.8%) and uveitis (16.4%). Patients with nr-axSpA and AS had similar EAM rates, disease activity, and QoL. Biologic disease-modifying anti-rheumatic drugs (bDMARDs; 83.2%) were the most commonly received medication, followed by conventional synthetic DMARDs (22.9%) and non-steroidal anti-inflammatory drugs (NSAIDs; 16.6%). At baseline, higher treatment satisfaction was reported with bDMARDs which, together with NSAIDs, were associated with the best overall scores for disease activity, function, and QoL in the overall population and AS subgroup.QUASAR is the first Italian prospective study that comprehensively evaluated a large axSpA patient sample in a real-world setting. This interim analysis at baseline confirmed that i) patients with AS and nr-axSpA have similar QoL and disease burden, ii) nearly all axSpA patients receive treatment, and iii) bDMARDs and NSAIDs, overall, yield better disease activity and QoL.
Published: 2018

45. NAILFOLD CAPILLAROSCOPY IN ANTISYNTHETASE SYNDROME (NASCAR): RESULTS OF A MULTICENTER, INTERNATIONAL STUDY OF THE AMERICAN AND EUROPEAN NETWORK OF ANTISYNTHETASE SYNDROME (AENEAS)

Author: Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, Ma, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, Ef, Castaneda, S, Selva-O'Callaghan, A, Araguas, Et, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, Cm, Longo, Fjl, Martinez-Barrio, J, Nieto-Gonzalez, Jc, Vichi, S, Confalonieri, M, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, Ca, Specker, C, Barsotti, S, Neri, R, Weinmann-Menke, J, Schwarting, A, Smith, V, and Cutolo, M
Published: 2018

46. P2x7 receptor in systemic lupus erythematosus (SLE). exploring a novel pathogenetic pathway in lupus related serositis

Author: Furini, F, Bortoluzzi, A, Giuliani, Al, Di Virgilio, F, and Govoni, M
Subjects: NO
Published: 2018

47. Development and first validation of a disease activity score for gout

Author: Scirè, Ca1, Carrara, G2, Viroli, C3, Cimmino, Ma4, Taylor, Wj5, Manara, M2, Govoni, M6, Salaffi, F7, Punzi, L8, Montecucco, C9, Matucci Cerinic M10, Minisola, G11, Collaborators Ariani A, Study Group for the Kick Off of the Italian Network for Gout S. t. u. d. y., Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabrò, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, Marco, Broggini, M, Caprioli, M, Favero, M, Sallì, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V., Scire, C, Carrara, G, Viroli, C, Cimmino, M, Taylor, W, Manara, M, Govoni, M, Salaffi, F, Punzi, L, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Scirè, Carlo A, Carrara, Greta, Viroli, Cinzia, Cimmino, Marco A., Taylor, William J., Manara, Maria, Govoni, Marcello, Salaffi, Fausto, Punzi, Leonardo, Montecucco, Carlomaurizio, Matucci-Cerinic, Marco, Minisola, Giovanni, Ariani, Alarico, Galossi, Alessandra, Lauriti, Ciro, Fracassi, Elena, Idolazzi, Luca, Bardelli, Marco, Selvi, Enrico, Tirri, Enrico, Furini, Federica, Inverardi, Flora, Calabrò, Andrea, Porta, Francesco, Bittelli, Raffaele, Venturino, Francesco, Capsoni, Franco, Prevete, Immacolata, Sebastiani, Giandomenico, Selmi, Carlo, Fabbriciani, Gianluigi, D'Avola, Giovanni, Botticella, Giulia, Serale, Francesca, Seminara, Giulia, D'Alessandro, Giuseppe, Santo, Leonardo, Longato, Lorena, Zaccara, Eleonora, Sinigaglia, Luigi, Atteritano, Marco, Broggini, Marco, Caprioli, Marta, Favero, Marta, Sallì, Salvatore, Scarati, Marco, Parisi, Simone, Malavolta, Nazzarena, Corvaglia, Stefania, Scarpato, Salvatore, and Veneto, Vittorio
Subjects: Male, medicine.medical_specialty, Visual analogue scale, Aged, Arthralgia, Factor Analysis, Statistical, Female, Follow-Up Studies, Gout, Humans, Joints, Linear Models, Middle Aged, Pain Measurement, Patient Reported Outcome Measures, Regression Analysis, Reproducibility of Results, Uric Acid, Disease Progression, Severity of Illness Index, NO, disease activity, gout, patient perspective, 03 medical and health sciences, 0302 clinical medicine, gout, Rheumatology, Discriminant function analysis, Linear regression, Criterion validity, medicine, 030212 general & internal medicine, 030203 arthritis & rheumatology, business.industry, Construct validity, Regression analysis, Statistical, medicine.disease, Linear discriminant analysis, patient perspective, Physical therapy, Rheumatology, Factor Analysis, business, Factor Analysis, disease activity
Abstract: Objective To develop a new composite disease activity score for gout and provide its first validation. Methods Disease activity has been defined as the ongoing presence of urate deposits that lead to acute arthritis and joint damage. Every measure for each Outcome Measures in Rheumatology core domain was considered. A 3-step approach (factor analysis, linear discriminant analysis, and linear regression) was applied to derive the Gout Activity Score (GAS). Decision to change treatment or 6-month flare count were used as the surrogate criteria of high disease activity. Baseline and 12-month followup data of 446 patients included in the Kick-Off of the Italian Network for Gout cohort were used. Construct- and criterion-related validity were tested. External validation on an independent sample is reported. Results Factor analysis identified 5 factors: patient-reported outcomes, joint examination, flares, tophi, and serum uric acid (sUA). Discriminant function analysis resulted in a correct classification of 79%. Linear regression analysis identified a first candidate GAS including 12-month flare count, sUA, visual analog scale (VAS) of pain, VAS global activity assessment, swollen and tender joint counts, and a cumulative measure of tophi. Alternative scores were also developed. The developed GAS demonstrated a good correlation with functional disability (criterion validity) and discrimination between patient- and physician-reported measures of active disease (construct validity). The results were reproduced in the external sample. Conclusion This study developed and validated a composite measure of disease activity in gout. Further testing is required to confirm its generalizability, responsiveness, and usefulness in assisting with clinical decisions.
Published: 2016

48. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

Author: González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, Cavagna, Lorenzo, González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, and Cavagna, Lorenzo
Abstract: Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility
Published: 2018

49. Lifestyle and dietary habits of patients with gout followed in rheumatology settings

Author: Manara, M, Carrara, G, Scire, C, Cimmino, M, Govoni, M, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Manara M., Carrara G., Scire C. A., Cimmino M. A., Govoni M., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., Veneto V., Manara, M, Carrara, G, Scire, C, Cimmino, M, Govoni, M, Montecucco, C, Matucci-Cerinic, M, Minisola, G, Ariani, A, Galossi, A, Lauriti, C, Fracassi, E, Idolazzi, L, Bardelli, M, Selvi, E, Tirri, E, Furini, F, Inverardi, F, Calabro, A, Porta, F, Bittelli, R, Venturino, F, Capsoni, F, Prevete, I, Sebastiani, G, Selmi, C, Fabbriciani, G, D'Avola, G, Botticella, G, Serale, F, Seminara, G, D'Alessandro, G, Santo, L, Longato, L, Zaccara, E, Sinigaglia, L, Atteritano, M, Broggini, M, Caprioli, M, Favero, M, Salli, S, Scarati, M, Parisi, S, Malavolta, N, Corvaglia, S, Scarpato, S, Veneto, V, Manara M., Carrara G., Scire C. A., Cimmino M. A., Govoni M., Montecucco C., Matucci-Cerinic M., Minisola G., Ariani A., Galossi A., Lauriti C., Fracassi E., Idolazzi L., Bardelli M., Selvi E., Tirri E., Furini F., Inverardi F., Calabro A., Porta F., Bittelli R., Venturino F., Capsoni F., Prevete I., Sebastiani G., Selmi C., Fabbriciani G., D'Avola G., Botticella G., Serale F., Seminara G., D'Alessandro G., Santo L., Longato L., Zaccara E., Sinigaglia L., Atteritano M., Broggini M., Caprioli M., Favero M., Salli S., Scarati M., Parisi S., Malavolta N., Corvaglia S., Scarpato S., and Veneto V.
Abstract: Diet and lifestyles modification are core aspects of the non-pharmacological management of gout, but a poor consistency with suggested guidelines is reported. This study aimed to investigate dietary and lifestyle habits of patients with gout followed in rheumatology settings. Data were retrieved from the baseline dataset of the KING study, a multicentre cohort study of patients with gout followed in rheumatology settings. Dietary habits were assessed with the Italian National Institute of Statistics (ISTAT) food-frequency questionnaire and compared with reported data about general population. The relative increase of exposure was estimated by standardized prevalence ratios adjusted for gender, age and geographical distribution. The study population included 446 patients, with a mean age of 63.9 years and a M/F ratio of 9:1. Compared to the Italian population, gouty patients showed a higher prevalence of obesity [1.82 (1.52-2.18)] and a higher consumption of wine [1.85 (1.48-2.32)] and beer [2.21 (1.68-2.90)], but a lower prevalence of smoking and a lower intake of liquor. They showed a lower intake of red meat [0.80 (0.71-0.91)], but a similar intake of other tested dietary factors. Gouty patients’ lifestyle is still partially different from the recommended.
Published: 2015

50. Clinical spectrum time course in anti jo-1 positive antisynthetase syndrome: Results from an international retrospective multicenter study

Author: Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Murcia, T, La Corte, R, Furini, F, Foschi, V, Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Montecucco, C, Gonzalez-Gay, M, Rosenthal, K, Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Giraldo W. A. S., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Murcia T. P., La Corte R., Furini F., Foschi V., Corral J. B., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Montecucco C., Gonzalez-Gay M. A., Rosenthal K., Cavagna, L, Nuno, L, Scire, C, Govoni, M, Longo, F, Franceschini, F, Neri, R, Castaneda, S, Giraldo, W, Caporali, R, Iannone, F, Fusaro, E, Paolazzi, G, Pellerito, R, Schwarting, A, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Bartoloni, E, Specker, C, Murcia, T, La Corte, R, Furini, F, Foschi, V, Corral, J, Airo, P, Cavazzana, I, Martinez-Barrio, J, Hinojosa, M, Giannini, M, Barsotti, S, Menke, J, Triantafyllias, K, Vitetta, R, Russo, A, Bajocchi, G, Bravi, E, Barausse, G, Bortolotti, R, Selmi, C, Parisi, S, Montecucco, C, Gonzalez-Gay, M, Rosenthal, K, Cavagna L., Nuno L., Scire C. A., Govoni M., Longo F. J. L., Franceschini F., Neri R., Castaneda S., Giraldo W. A. S., Caporali R., Iannone F., Fusaro E., Paolazzi G., Pellerito R., Schwarting A., Saketkoo L. A., Ortego-Centeno N., Quartuccio L., Bartoloni E., Specker C., Murcia T. P., La Corte R., Furini F., Foschi V., Corral J. B., Airo P., Cavazzana I., Martinez-Barrio J., Hinojosa M., Giannini M., Barsotti S., Menke J., Triantafyllias K., Vitetta R., Russo A., Bajocchi G., Bravi E., Barausse G., Bortolotti R., Selmi C., Parisi S., Montecucco C., Gonzalez-Gay M. A., and Rosenthal K.
Abstract: Anti Jo-1 antibodies are the main markers of the anti-synthetase syndrome (ASSD), an autoimmune disease clinically characterized by the occurrence of arthritis, myositis, and interstitial lung disease (ILD). These manifestations usually co-occur (for practical purpose complete forms) in the same patient, but cases with only 1 or 2 of these findings (for practical purpose incomplete forms) have been described. In incomplete forms, the ex novo occurrence of further manifestations is possible, although with frequencies and timing not still defined. The aim of this international, multicenter, retrospective study was to characterize the clinical time course of anti Jo-1 positive ASSD in a large cohort of patients. Included patients should be anti Jo-1 positive and with at least 1 feature between arthritis, myositis, and ILD. We evaluated the differences between complete and incomplete forms, timing of clinical picture appearance and analyzed factors predicting the appearance of further manifestations in incomplete ASSD. Finally, we collected 225 patients (58 males and 167 females) with a median follow-up of 80 months. At the onset, complete ASSD were 44 and incomplete 181. Patients with incomplete ASSD had frequently only 1 of the classic triad findings (110 cases), in particular, isolated arthritis in 54 cases, isolated myositis in 28 cases, and isolated ILD in 28 cases. At the end of follow-up, complete ASSD were 113, incomplete 112. Only 5 patients had an isolated arthritis, only 5 an isolated myositis, and 15 an isolated ILD. During the follow-up, 108 patients with incomplete forms developed further manifestations. Single main feature onset was the main risk factor for the ex novo appearance of further manifestation. ILD was the prevalent ex novo manifestation (74 cases). In conclusion, ASSD is a condition that should be carefully considered in all patients presenting with arthritis, myositis, and ILD, even when isolated. The ex novo appearance of further manifestati
Published: 2015

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