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1. 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

2. 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

3. 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

4. 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

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, 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

6. 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

7. 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

8. 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

9. Intravenous methylprednisolone pulses in hospitalised patients with severe COVID-19 pneumonia, A double-blind, randomised, placebo-controlled trial

Author: Salvarani, C, Massari, M, Costantini, M, Franco Merlo, D, Lucia Mariani, G, Viale, P, Nava, S, Guaraldi, G, Dolci, G, Boni, L, Savoldi, L, Bruzzi, P, Turrà, C, Catanoso, M, Maria Marata, A, Barbieri, C, Valcavi, A, Franzoni, F, Cavuto, S, Mazzi, G, Corsini, R, Trapani, F, Bartoloni, A, Barisione, E, Jole Burastero, G, Pan, A, Inojosa, W, Scala, R, Burattini, C, Luppi, F, Codeluppi, M, Eldin Tarek, K, Cenderello, G, Salio, M, Foti, G, Dongilli, R, Bajocchi, G, Alberto Negri, E, Ciusa, G, Fornaro, G, Bassi, I, Zammarchi, L, Aloè, T, Facciolongo, N, Salvarani, Carlo, Massari, Marco, Costantini, Massimo, Franco Merlo, Domenico, Lucia Mariani, Gabriella, Viale, Pierluigi, Nava, Stefano, Guaraldi, Giovanni, Dolci, Giovanni, Boni, Luca, Savoldi, Luisa, Bruzzi, Paolo, Turrà, Caterina, Catanoso, Mariagrazia, Maria Marata, Anna, Barbieri, Chiara, Valcavi, Annamaria, Franzoni, Francesca, Cavuto, Silvio, Mazzi, Giorgio, Corsini, Romina, Trapani, Fabio, Bartoloni, Alessandro, Barisione, Emanuela, Jole Burastero, Giulia, Pan, Angelo, Inojosa, Walter, Scala, Raffaele, Burattini, Cecilia, Luppi, Fabrizio, Codeluppi, Mauro, Eldin Tarek, Kamal, Cenderello, Giovanni, Salio, Mario, Foti, Giuseppe, Dongilli, Roberto, Bajocchi, Gianluigi, Alberto Negri, Emanuele, Ciusa, Giacomo, Fornaro, Giacomo, Bassi, Ilaria, Zammarchi, Lorenzo, Aloè, Teresita, Facciolongo, Nicola, Salvarani, C, Massari, M, Costantini, M, Franco Merlo, D, Lucia Mariani, G, Viale, P, Nava, S, Guaraldi, G, Dolci, G, Boni, L, Savoldi, L, Bruzzi, P, Turrà, C, Catanoso, M, Maria Marata, A, Barbieri, C, Valcavi, A, Franzoni, F, Cavuto, S, Mazzi, G, Corsini, R, Trapani, F, Bartoloni, A, Barisione, E, Jole Burastero, G, Pan, A, Inojosa, W, Scala, R, Burattini, C, Luppi, F, Codeluppi, M, Eldin Tarek, K, Cenderello, G, Salio, M, Foti, G, Dongilli, R, Bajocchi, G, Alberto Negri, E, Ciusa, G, Fornaro, G, Bassi, I, Zammarchi, L, Aloè, T, Facciolongo, N, Salvarani, Carlo, Massari, Marco, Costantini, Massimo, Franco Merlo, Domenico, Lucia Mariani, Gabriella, Viale, Pierluigi, Nava, Stefano, Guaraldi, Giovanni, Dolci, Giovanni, Boni, Luca, Savoldi, Luisa, Bruzzi, Paolo, Turrà, Caterina, Catanoso, Mariagrazia, Maria Marata, Anna, Barbieri, Chiara, Valcavi, Annamaria, Franzoni, Francesca, Cavuto, Silvio, Mazzi, Giorgio, Corsini, Romina, Trapani, Fabio, Bartoloni, Alessandro, Barisione, Emanuela, Jole Burastero, Giulia, Pan, Angelo, Inojosa, Walter, Scala, Raffaele, Burattini, Cecilia, Luppi, Fabrizio, Codeluppi, Mauro, Eldin Tarek, Kamal, Cenderello, Giovanni, Salio, Mario, Foti, Giuseppe, Dongilli, Roberto, Bajocchi, Gianluigi, Alberto Negri, Emanuele, Ciusa, Giacomo, Fornaro, Giacomo, Bassi, Ilaria, Zammarchi, Lorenzo, Aloè, Teresita, and Facciolongo, Nicola
Abstract: Rationale Pulse glucocorticoid therapy is used in hyperinflammation related to coronavirus disease 2019 (COVID-19). We evaluated the efficacy and safety of pulse intravenous methylprednisolone in addition to standard treatment in COVID-19 pneumonia. Methods In this multicentre, randomised, double-blind, placebo-controlled trial, 304 hospitalised patients with COVID-19 pneumonia were randomised to receive 1 g of methylprednisolone intravenously for three consecutive days or placebo in addition to standard dexamethasone. The primary outcome was the duration of patient hospitalisation, calculated as the time interval between randomisation and hospital discharge without the need for supplementary oxygen. The key secondary outcomes were survival free from invasive ventilation with orotracheal intubation and overall survival. Results Overall, 112 (75.4%) out of 151 patients in the pulse methylprednisolone arm and 111 (75.2%) of 150 in the placebo arm were discharged from hospital without oxygen within 30 days from randomisation. Median time to discharge was similar in both groups (15 days, 95% CI 13.0–17.0 days and 16 days, 95% CI 13.8–18.2 days, respectively; hazard ratio (HR) 0.92, 95% CI 0.71–1.20; p=0.528). No significant differences between pulse methylprednisolone and placebo arms were observed in terms of admission to intensive care unit with orotracheal intubation or death (20.0% versus 16.1%; HR 1.26, 95% CI 0.74–2.16; p=0.176) or overall mortality (10.0% versus 12.2%; HR 0.83, 95% CI 0.42–1.64; p=0.584). Serious adverse events occurred with similar frequency in the two groups. Conclusions Methylprenisolone pulse therapy added to dexamethasone was not of benefit in patients with COVID-19 pneumonia.
Published: 2022

10. AB0722 One-year clinical experience on the use of Nintedanib in Systemic Sclerosis: safety profile.

Author: Magnani, L., primary, Spinella, A., additional, Testoni, S., additional, Bajocchi, G., additional, Dardani, L., additional, Salvarani, C., additional, and Giuggioli, D., additional
Published: 2022
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11. AB0725 Scleroderma study group Emilia Romagna (Sclero-RER): real life use of prostacyclin analog. Preliminary data from a multicentric survey.

Author: Magnani, L., primary, Ariani, A., additional, Girelli, F., additional, Spinella, A., additional, Lumetti, F., additional, Lo Monaco, A., additional, Reta, M., additional, Arrigoni, E., additional, Ursini, F., additional, Bezzi, A., additional, Cataleta, P., additional, Montaguti, L., additional, Trevisani, M., additional, Colina, M., additional, Bernardi, S., additional, Becciolini, A., additional, Galoppini, G., additional, Pignataro, F., additional, Ciaffi, J., additional, Bravi, E., additional, Focherini, M. C., additional, Moscatelli, S., additional, Sambo, P., additional, Mule’, R., additional, Corvaglia, S., additional, Bajocchi, G., additional, Conti, D., additional, Salvarani, C., additional, and Giuggioli, D., additional
Published: 2022
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12. GENDER AND FIBROMYALGIA SEVERITY: REAL LIFE DATA FROM THE ITALIAN REGISTRY

Author: Batticciotto, A, Campanaro, F, Atzeni, F, Alciati, A, Di Carlo, M, Bazzichi, L, Govoni, M, Biasi, G, Di Franco, M, Mozzani, F, Gremese, E, Dagna, L, Fischetti, F, Giacomelli, R, Guiducci, S, Guggino, G, Bentivegna, M, Gerli, R, Salvarani, C, Bajocchi, G, Ghini, M, Iannone, F, Giorgi, V, Farah, S, Bonazza, S, Barbagli, S, Gioia, C, Capacci, A, Cavalli, G, Carubbi, F, Nacci, F, Ilenia, R, Sinigaglia, L, Cutolo, M, Cappelli, A, Sarzi-Puttini, P, Salaffi, F, Batticciotto, A, Campanaro, F, Atzeni, F, Alciati, A, Di Carlo, M, Bazzichi, L, Govoni, M, Biasi, G, Di Franco, M, Mozzani, F, Gremese, E, Dagna, L, Fischetti, F, Giacomelli, R, Guiducci, S, Guggino, G, Bentivegna, M, Gerli, R, Salvarani, C, Bajocchi, G, Ghini, M, Iannone, F, Giorgi, V, Farah, S, Bonazza, S, Barbagli, S, Gioia, C, Capacci, A, Cavalli, G, Carubbi, F, Nacci, F, Ilenia, R, Sinigaglia, L, Cutolo, M, Cappelli, A, Sarzi-Puttini, P, and Salaffi, F
Subjects: Fibromyalgia, Rheumatology
Abstract: Background: Fibromyalgia (FM) patients report chronic widespread pain, fatigue, cognitive difficulties and sleep disturbances, often associated with anxiety and/or depression (1). FM syndrome more frequently affects women and many papers describe gender-related differences in the perception, description and expression of pain (2), but up to now, the impact of gender on the clinical severity of FM is still a controversial topic. Objectives: The aim of this study was to analyse the data from a web-based registry of FM patients in order to detect a relationship between gender and disease severity. Methods: Adult patients with FM, diagnosed on the basis of the 2010/2011 American College of Rheumatology (ACR) diagnostic criteria (3), were recruited at 19 Italian rheumatology centres between November 2018 and April 2019. Those affected by other conditions that could interfere with the assessment of FM, e.g. psychiatric disorders, were excluded from the study. The severity of the disease was evaluated by validated FM-specific questionnaires: the revised Fibromyalgia Impact Questionnaire (FIQR) (4), the modified Fibromyalgia Assessment Status (ModFAS) questionnaire (5), and the Polysymptomatic Distress Scale (PDS) (6). The data obtained were collected in the Italian Fibromyalgia Registry, an online registry created with the support of the Italian Society of Rheumatology (SIR). Results: We analyse data from 2.381 patients affected by FM, 2.184 females (91.7%) and 197 males. No significant differences in mean age, disease duration, or BMI between the two genders were reported. The women expressed greater disease burden as indicated by higher scores for each completed test: higher mean ModFAS score (25.23 ± 8.83 Vs 23.37 ± 9.22; p = 0.005), mean FIQR score (58.62 ± 23.22 Vs 51.68 ± 23.06; p
Published: 2021

13. Long-term methotrexate use in Rheumatoid Arthritis patients: real-world data from the MARTE study

Author: Serban, T., Allara, R., Azzolini, V., Bellintani, C., Belloli, L., Belai Beyene, N., Bucci, R., Caporali, R., Cappelli, A., Corbelli, V., de Gennaro, F., Fusaro, E., Giusti, A., Govoni, M., Magnani, L., Manzo, C., Romano, C., Rossini, M., Santilli, D., Savi Ola, G., Sinigaglia, L., Bianchi, G., Arnoldi, C., Arrigoni, E., Bajocchi, G., Beccaris, A., Brussino, L., Califano, E., Carlino, G., Castellana, P., Del Piano, M., Delvino, P. G., Denotti, A., Diana, P., Epis, O. M., Fava Lli, E., Foti, R., Ghiringhelli, P., Gilardi, A. G., Iagnocco, A., Idolazzi, L., Italiano, G., Lapadula, G., Lomater, C., Longhi, M., Lupo, A., Malav Olta, N., Manara, M., Marchetta, A., Marcialis, M. R., Mathieu, A., Mazzochi, D., Mosca, M., Muratore, M., Naclerio, C., Nallino, G., Nutile, G., Pendolino, M., Piccolo, S., Ricioppo, A., Romeo, N., Rossini, T., Salvatore, S., Sambataro, A., Sangari, D., Santo, L., Selmi, C. F., Semeraro, A., Serafino, L., Tartarelli, G., Tirri, E., Todoerti, M., Traballi, G., Tropea, S., Zizo, G., Zuccaro, C., Serban, Teodora, Allara, Roberto, Azzolini, Valeria, Bellintani, Claudio, Belloli, Laura, Belai Beyene, Nebiat, Bucci, Romano, Caporali, Roberto, Cappelli, Antonella, Corbelli, Vincenzo, DE Gennaro, Fabio, Fusaro, Enrico, Giusti, Andrea, Govoni, Marcello, Magnani, Luca, Manzo, Ciro, Romano, Ciro, Rossini, Maurizio, Santilli, Daniele, Saviola, Gianantonio, Sinigaglia, Luigi, and Bianchi, Gerolamo
Subjects: Male, rheumatoid arthritis, Time Factors, Cross-sectional study, Arthritis, Arthritis, Rheumatoid, 0302 clinical medicine, Methotrexate, Arthritis, rheumatoid, Long-term care, Citizen science, Intramuscular, Smokers, Subcutaneous, marte study, General Medicine, Postmenopause, Italy, 030220 oncology & carcinogenesis, Rheumatoid arthritis, Antirheumatic Agents, 030211 gastroenterology & hepatology, Female, medicine.drug, musculoskeletal diseases, medicine.medical_specialty, rheumatoid, Injections, Subcutaneous, Injections, Intramuscular, methotrexate, Injections, NO, 03 medical and health sciences, Route of administration, Sex Factors, Internal medicine, medicine, Humans, Dosing, Aged, Rheumatoid, Cross-Sectional Studies, Socioeconomic Factors, rheumatoid arthritis, marte study, methotrexate, business.industry, medicine.disease, Rheumatology, Discontinuation, business
Abstract: BACKGROUND The MARTE study investigated the demographic, clinical, and therapeutic characteristics of rheumatoid arthritis (RA) patients ongoing methotrexate (MTX) treatment for longer than 8 years. METHODS This cross-sectional, observational study considered 587 RA patients from 67 Rheumatology Units across Italy. Data collected included demographic, clinical, and therapeutic characteristics, focusing on MTX prescription patterns (route of administration, dosing regimens, treatment duration, and discontinuation). RESULTS As initial therapy, 90.6% of patients received one conventional synthetic Disease Modifying Anti Rheumatic Drug (csDMARD), with treatment started within the first 3 months from diagnosis in half of the patients. MTX was the first csDMARD in 46.2% of patients. The prevalent route of administration at diagnosis was the intramuscular (60.5%), while at study entry (baseline) 57.6% were receiving subcutaneous MTX. Patients who required a higher MTX dose at study entry were those who received a significantly lower starting MTX dose (P
Published: 2021

14. Definition of fibromyalgia severity: Findings from a cross-sectional survey of 2339 Italian patients

Author: Salaffi, F., Di Carlo, M., Bazzichi, L., Atzeni, F., Govoni, M., Biasi, G., Di Franco, M., Mozzani, F., Gremese, E., Dagna, L., Batticciotto, A., Fischetti, F., Giacomelli, R., Guiducci, S., Guggino, G., Bentivegna, M., Gerli, R., Salvarani, C., Bajocchi, G., Ghini, M., Iannone, F., Giorgi, V., Farah, S., Cirillo, M., Bonazza, S., Barbagli, S., Gioia, C., Santilli, D., Capacci, A., Cavalli, G., Carubbi, F., Nacci, F., Riccucci, I., Sinigaglia, L., Masullo, M., Polizzi, B. M., Cutolo, M., Sarzi-Puttini, P., Gremese E. (ORCID:0000-0002-2248-1058), Bentivegna M., Santilli D., Cavalli G. (ORCID:0000-0003-1968-8987), Masullo M., Salaffi, F., Di Carlo, M., Bazzichi, L., Atzeni, F., Govoni, M., Biasi, G., Di Franco, M., Mozzani, F., Gremese, E., Dagna, L., Batticciotto, A., Fischetti, F., Giacomelli, R., Guiducci, S., Guggino, G., Bentivegna, M., Gerli, R., Salvarani, C., Bajocchi, G., Ghini, M., Iannone, F., Giorgi, V., Farah, S., Cirillo, M., Bonazza, S., Barbagli, S., Gioia, C., Santilli, D., Capacci, A., Cavalli, G., Carubbi, F., Nacci, F., Riccucci, I., Sinigaglia, L., Masullo, M., Polizzi, B. M., Cutolo, M., Sarzi-Puttini, P., Gremese E. (ORCID:0000-0002-2248-1058), Bentivegna M., Santilli D., Cavalli G. (ORCID:0000-0003-1968-8987), and Masullo M.
Abstract: Objective: To establish optimal cut-off values for the scores of the revised Fibromyalgia Impact Questionnaire (FIQR), the modified Fibromialgia Assessment Scale (FAS 2019mod), and the Polysymptomatic Distress Scale (PDS) in order to distinguish five levels of FM disease severity. Methods: Consecutive FM patients were evaluated with the three clinimetric indices, and each patient was required to answer the anchor question: 'In general, would you say your health is 1 = very good, 2 = good, 3 = fair, 4 = poor, or 5 = very poor?'-which represented the external criterion. Cut-off points were established through the interquartile reconciliation approach. Results: The study sample consisted of 2181 women (93.2%) and 158 men (6.8%), with a mean age of 51.9 (11.5) years, and mean disease duration was 7.3 (6.9) years. The overall median FIQR, FAS 2019 mod and PDS scores (25th-75th percentiles) were respectively 61.16 (41.16-77.00), 27.00 (19.00-32.00) and 19.0 (13.00-24.00). Reconciliation of the mean 75th and 25th percentiles of adjacent categories defined the severity states for FIQR: 0-23 for remission, 24-40 for mild disease, 41-63 for moderate disease, 64-82 for severe disease and >83 for very severe disease; FAS 2019 mod: 0-12 for remission, 13-20 for mild disease, 21-28 for moderate disease, 29-33 for severe disease and >33 for very severe disease; PDS: 0-5 for remission, 6-15 for mild disease, 16-20 for moderate disease, 21-25 for severe disease and >25 for very severe disease. Conclusions: Disease severity cut-offs can represent an important improvement in interpreting FM.
Published: 2021

15. AB0716 FIBROMYALGIA SYNDROME SEVERITY ACCORDING TO AGE CATEGORIES: RESULTS FROM A NATIONAL REGISTER

Author: Di Carlo, M., primary, Farah, S., additional, Bazzichi, L., additional, Atzeni, F., additional, Govoni, M., additional, Biasi, G., additional, DI Franco, M., additional, Mozzani, F., additional, Gremese, E., additional, Dagna, L., additional, Batticciotto, A., additional, Fischetti, F., additional, Giacomelli, R., additional, Guiducci, S., additional, Guggino, G., additional, Bentivegna, M., additional, Gerli, R., additional, Salvarani, C., additional, Bajocchi, G., additional, Ghini, M., additional, Iannone, F., additional, Giorgi, V., additional, Cirillo, M., additional, Bonazza, S., additional, Barbagli, S., additional, Gioia, C., additional, Marino, N. G., additional, Capacci, A., additional, Cavalli, G., additional, Cappelli, A., additional, Carubbi, F., additional, Nacci, F., additional, Ilenia, R., additional, Cutolo, M., additional, Sinigaglia, L., additional, Sarzi-Puttini, P., additional, and Salaffi, F., additional
Published: 2021
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16. OP0310 GENDER AND FIBROMYALGIA SEVERITY: REAL LIFE DATA FROM THE ITALIAN REGISTRY

Author: Batticciotto, A., primary, Campanaro, F., additional, Atzeni, F., additional, Alciati, A., additional, DI Carlo, M., additional, Bazzichi, L., additional, Govoni, M., additional, Biasi, G., additional, DI Franco, M., additional, Mozzani, F., additional, Gremese, E., additional, Dagna, L., additional, Fischetti, F., additional, Giacomelli, R., additional, Guiducci, S., additional, Guggino, G., additional, Bentivegna, M., additional, Gerli, R., additional, Salvarani, C., additional, Bajocchi, G., additional, Ghini, M., additional, Iannone, F., additional, Giorgi, V., additional, Farah, S., additional, Bonazza, S., additional, Barbagli, S., additional, Gioia, C., additional, Capacci, A., additional, Cavalli, G., additional, Carubbi, F., additional, Nacci, F., additional, Ilenia, R., additional, Sinigaglia, L., additional, Cutolo, M., additional, Cappelli, A., additional, Sarzi-Puttini, P., additional, and Salaffi, F., additional
Published: 2021
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17. 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

18. 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

19. Neurological Involvement in Primary Sjo¨gren’s Syndrome: Clinical and Instrumental Evaluation in a Cohort of Italian Patients

Author: Govoni, M., Bajocchi, G., Rizzo, N., Tola, M. R., Caniatti, L., Tugnoli, V., Colamussi, P., and Trotta, F.
Published: 1999
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20. SAT0338 CONTRAST-ENHANCED ULTRASONOGRAPHY IN THE EVALUATION OF MYOSITIS

Author: Pipitone, N., primary, Macchioni, P., additional, Bajocchi, G., additional, and Salvarani, C., additional
Published: 2020
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21. 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

22. Risk factors for severe cranial ischaemic events in an Italian population-based cohort of patients with giant cell arteritis

Author: Salvarani, C., Bella, C. Della, Cimino, L., Macchioni, P., Formisano, D., Bajocchi, G., Pipitone, N., Catanoso, M. G., Restuccia, G., Ghinoi, A., and Boiardi, L.
Published: 2009

23. Pre-pregnancy counselling of patients with vasculitis

Author: Doria, A., Bajocchi, G., Tonon, M., and Salvarani, C.
Published: 2008

24. Cervical interspinous bursitis in active polymyalgia rheumatica

Author: Salvarani, C, Barozzi, L, Cantini, F, Niccoli, L, Boiardi, L, Valentino, M, Pipitone, N, Bajocchi, G, Macchioni, P, Catanoso, M G, Olivieri, I, and Hunder, G G
Published: 2008
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25. –463 G/A myeloperoxidase promoter polymorphism in giant cell arteritis

Author: Salvarani, C, Casali, B, Farnetti, E, Pipitone, N, Nicoli, D, Macchioni, P L, Cimino, L, Bajocchi, G L, Catanoso, M G, Pattacini, L, Ghinoi, A, Restuccia, G, and Boiardi, L
Published: 2008
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26. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author: Elhai, M, Boubaya, M, Distler, O, Smith, V, Matucci-Cerinic, M, Sancho, JJ, Truchetet, ME, Braun-Moscovici, Y, Iannone, F, Novikov, PI, Lescoat, A, Siegert, E, Castellvi, I, Airo, P, Vettori, S, Langhe, E, Hachulla, E, Erler, A, Ananieva, L, Krusche, M, Lopez-Longo, FJ, Distler, JHW, Hunzelmann, N, Hoffmann-Vold, AM, Riccieri, V, Hsu, VM, Pozzi, MR, Ancuta, C, Rosato, E, Mihai, C, Kuwana, M, Saketkoo, LA, Chizzolini, C, Hesselstrand, R, Ullman, S, Yavuz, S, Rednic, S, Caimmi, C, Bloch-Queyrat, C, Allanore, Y, Guiducci, S, Walker, UA, Kyburz, D, Lapadula, G, Maurer, B, Jordan, S, Dobrota, R, Becvar, R, Sierakowsky, S, Bielecka, OK, Sulli, A, Cutolo, M, Cuomo, G, Nicoara, I, Kahan, A, Vlachoyiannopoulos, PG, Montecucco, CM, Caporali, R, Stork, J, Inanc, M, Carreira, PE, Novak, S, Czirjak, L, Varju, C, Kucharz, EJ, Kotulska, A, Kopec-Medrek, M, Widuchowska, M, Cozzi, F, Rozman, B, Mallia, C, Coleiro, B, Gabrielli, A, Farge, D, Wu, C, Marjanovic, Z, Faivre, H, Hij, D, Dhamadi, R, Wollheim, F, Scheja, A, Wuttge, DM, Andreasson, K, Martinovic, D, Balbir-Gurman, A, Trotta, F, Lo Monaco, A, Pellerito, R, Mauriziano, O, Caramaschi, P, Morovic-Vergles, J, Black, C, Denton, C, Damjanov, N, Henes, J, Santamaria, VO, Heitmann, S, Krasowska, D, Matthias, Hasler, P, Burkhardt, H, Himsel, A, Bajocchi, G, Da Silva, JAP, Salvador, MJ, Stamenkovic, B, Stankovic, A, Selmi, CF, De Santis, M, Tikly, M, Denisov, LN, Herrick, A, Muller-Ladner, U, Frerix, M, Tarner, I, Scorza, R, Puppo, F, Engelhart, M, Strauss, G, Nielsen, H, Damgaard, K, Szucs, G, Mendoza, AZ, de la Puente, C, Giraldo, WAS, Midtvedt, O, Reiseter, S, Garen, T, Launay, D, Valesini, G, Ionescu, RM, Groseanu, L, Opris, D, Cornateanu, RS, Ionitescu, R, Gherghe, AM, Soare, A, Gorga, M, Bojinca, M, Milicescu, M, Sunderkotter, C, Kuhn, A, Sandorfi, N, Schett, G, Beyer, C, Meroni, P, Ingegnoli, F, Mouthon, L, De Keyser, F, Melsens, K, Cantatore, FP, Corrado, A, Iversen, L, von Muhlen, CA, Bohn, JM, Lonzetti, LS, Eyerich, K, Hein, R, Knott, E, Wiland, P, Szmyrka-Kaczmarek, M, Sokolik, R, Morgiel, E, Madej, M, Houssiau, FA, Krummel-Lorenz, B, Saar, P, Aringer, M, Gunther, C, Westhovens, R, Lenaerts, J, Anic, B, Baresic, M, Mayer, M, Uprus, M, Otsa, K, Granel, B, Muller, CD, Radominski, SC, Azevedo, VF, Jimenez, S, Busquets, J, Agachi, S, Groppa, L, Chiaburu, L, Russu, E, Popa, S, Zenone, T, Pileckyte, M, Mathieu, A, Vacca, A, Sampaio-Barros, PD, Yoshinari, NH, Marangoni, RG, Martin, P, Fuocco, L, Stebbings, S, Highton, J, Chapman, P, O'Donnell, J, Stamp, L, Doube, A, Solanki, K, Veale, D, O'Rourke, M, Loyo, E, Li, MT, Mohamed, WAAA, Amoroso, A, Gigante, A, Oksel, F, Yargucu, F, Tanaseanu, CM, Popescu, M, Dumitrascu, A, Tiglea, I, Foti, R, Chirieac, R, Furst, D, Villiger, P, Adler, S, van Laar, J, Kayser, C, Fathi, N, Hassanien, M, Lefebvre, PGD, Rubio, SR, Exposito, MV, Chatelus, E, Sibilia, J, Gottenberg, JE, Chifflot, H, Litinsky, I, Emery, P, Buch, M, Del Galdo, F, Venalis, A, Butrimiene, I, Venalis, P, Rugiene, R, Karpec, D, Lasky, JA, Cosentino, V, Kerzberg, E, Montoya, F, Bianchi, W, Carneiro, S, Maretti, GB, Bianchi, DV, Limonta, M, Lupi, ALBE, Lupi, E, Rosner, I, Rozenbaum, M, Slobodin, G, Boulman, N, Rimar, D, Couto, M, Kahl, S, Chen, F, McCloskey, D, Malveaux, H, Spertini, F, Ribi, C, Buss, G, Martin, T, Guffroy, A, Poindron, V, Chotchaeva, F, Mukhin, NA, Moiseev, S, EUSTAR Network, Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan José, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I, Lescoat, Alain, Siegert, Elise, Castellví, Ivan, Airó, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, López-Longo, F. J., Distler, Jörg H W, Hunzelmann, Nicola, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M, Pozzi, Maria R, Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, Kuwana, Masataka, Saketkoo, Lesley Ann, Chizzolini, Carlo, Hesselstrand, Roger, Ullman, Susanne, Yavuz, Sule, Rednic, Simona, Caimmi, Cristian, Bloch-Queyrat, Coralie, Allanore, Yannick, and Cuomo, Giovanna
Subjects: Male, Vital capacity, systemic sclerosis, Pulmonary Fibrosis, Vital Capacity, Scleroderma, lung fibrosis, rituximab, skin fibrosis, immune system diseases, DLCO, hemic and lymphatic diseases, Immunology and Allergy, Medicine, Prospective Studies, Registries, skin and connective tissue diseases, Prospective cohort study, Lung, skin fibrosi, Skin, ddc:616, integumentary system, Orvostudományok, Middle Aged, Respiratory Function Tests, lung fibrosis, rituximab, skin fibrosis, systemic sclerosis, Treatment Outcome, lung fibrosi, Antirheumatic Agents, Systemic sclerosis, Rituximab, Female, systemic sclerosi, medicine.drug, Adult, medicine.medical_specialty, Immunology, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, FEV1/FVC ratio, Rheumatology, Internal medicine, Humans, Adverse effect, Propensity Score, Aged, Biochemistry, Genetics and Molecular Biology (all), Scleroderma, Systemic, Skin fibrosis, business.industry, medicine.disease, Fibrosis, Lung fibrosis, business
Abstract: ObjectiveTo assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice.MethodsWe performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab.Results254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47–5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55–1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56–3.53], pConclusionRituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.
Published: 2019

27. Susceptibility and severity of COVID-19 in patients treated with bDMARDS and tsDMARDs: a population-based study

Author: Salvarani, C., Bajocchi, G., Mancuso, P., Galli, E., Muratore, F., Boiardi, L., Catanoso, M., Pipitone, N., Cassone, G., Girolimetto, N., Croci, S., Cimino, L., Gradellini, F., Beltrami, M., Di Lernia, V., Dolci, G., Massari, M., Marata, A. M., Costantini, M., and Giorgi Rossi, P.
Subjects: Male, 0301 basic medicine, Severity of Illness Index, 0302 clinical medicine, Piperidines, Risk Factors, Epidemiology, Immunology and Allergy, media_common, Sulfonamides, education.field_of_study, Middle Aged, Hospitalization, Antirheumatic Agents, Italy, Female, Ustekinumab, Disease Susceptibility, Coronavirus Infections, autoimmune diseases, biological therapy, epidemiology, Abatacept, Adult, Aged, Antibodies, Monoclonal, Humanized, Azetidines, Betacoronavirus, Humans, Interleukin 1 Receptor Antagonist Protein, Janus Kinase Inhibitors, Logistic Models, Pandemics, Pneumonia, Viral, Proportional Hazards Models, Pyrimidines, Pyrroles, Rheumatic Diseases, Tumor Necrosis Factor Inhibitors, medicine.drug, Drug, medicine.medical_specialty, media_common.quotation_subject, Immunology, Population, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Rheumatology, Internal medicine, Severity of illness, medicine, education, 030203 arthritis & rheumatology, SARS-CoV-2, business.industry, Proportional hazards model, COVID-19, 030104 developmental biology, Purines, Pyrazoles, business
Abstract: Patients with autoimmune conditions treated with biological agents have an increased risk of severe infections.1 2 Very few studies have evaluated the susceptibility and severity of coronavirus disease 2019 (COVID-19) in patients treated with biological disease-modifying antirheumatic drugs (bDMARDs) or targeted synthetic DMARDs (tsDMARDs).3 4 Some of these studies suggest a protective role of these drugs for COVID-19; however, they consist of small series, and the results are unclear. Therefore, we decided to evaluate in a population-based study the risk of COVID-19 infection and its severity in the patients treated with bDMARDs or tsDMARDs in a geographic area (Emilia Romagna) at high diffusion of COVID-19. We identified 1195 patients treated with the bDMARDs or tsDMARDs listed in table 1 in Reggio Emilia area on 31 December 2019. Biological agents were classified according to the mechanism of action. The patients were registered in the database of the Hospital Pharmaceutical Service of the Reggio Emilia area, which delivers the drug directly to the patients. The database is updated every 3 months. All residents …
Published: 2020

28. 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

29. Cerebral hypoperfusion detected by SPET in early neuro-Behçet syndrome

Author: TROTTA, F., BAJOCCHI, G., COLAMUSSI, P., SANDRI, G., CIANCIO, G., TOLA, M. R., MENEGALE, G., CITTANTI, C., GIGANTI, M., and PIFFANELLI, A.
Published: 1998

30. Brain perfusion SPET and proton magnetic resonance spectroscopy in the evaluation of two systemic lupus erythematosus patients with mild neuropsychiatric manifestations

Author: COLAMUSSI, P., TROTTA, F., RICCI, R., CITTANTI, C., GOVONI, M., BARBARELLA, G., GIGANTI, M., BAJOCCHI, G., UCCELLI, L., TREVISAN, C., and PIFFANELLI, A.
Published: 1997

31. 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

32. Histopathology of vasculitis

Author: Bajocchi, G., primary and Cavazza, A., additional
Published: 2018
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33. AB0782 Systemic sclerosis patients with concomitant psoriasis: a proof-of-concept pilot study

Author: Magnani, L., primary, Bajocchi, G., additional, Chiarolanza, I., additional, CAruso, A., additional, Aldigeri, R., additional, and Salvarani, C., additional
Published: 2018
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34. Nerve ablation after bronchial thermoplasty and sustained improvement in severe asthma

Author: Facciolongo, N., primary, Di Stefano, A., additional, Pietrini, V., additional, Galeone, C., additional, Bellanova, F., additional, Menzella, F., additional, Scichilone, N., additional, Piro, R., additional, Bajocchi, G. L., additional, Balbi, B., additional, Agostini, L., additional, Salsi, P. P., additional, Formisano, D., additional, and Lusuardi, M., additional
Published: 2018
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35. Brief report: Successful pregnancies but a higher risk of preterm births in systemic sclerosis: An Italian multicentric study

Author: Taraborelli M, Ramoni V, Brucato A, Airò P, Bajocchi G, Bellisai F, Biasi D, Blagojevic J, Canti V, Caporali R, Caramaschi P, Chiarolanza I, Codullo V, Cozzi F, Cutolo M, De Santis M, De Vita S, Di Poi E, Doria A, Faggioli P, Favaro M, Ferraccioli G, Ferri C, Foti R, Gerosa A, Gerosa M, Giacuzzo S, GianiL, Giuggioli D, Imazio M, Iudici M, Iuliano A, Leonardi R, Limonta M, Lojacono A, Lubatti C, Matucci Cerinic M, Mazzone A, Meroni M, Meroni PL, Mosca M, Motta M, Muscarà M, Nava S, Padovan M, Pagani G, Paolazzi G, Peccatori S, Ravagnani V, Riccieri V, Rosato E, Rovere Querini P, Salsano F, Santaniello A, Scorza R, Tani C, Valesini G, Vanoli M, Vigone B, Zeni S, Tincani A, on behalf ofthe IMPRESS Investigators, CUOMO, Giovanna, VALENTINI, Gabriele, Taraborelli, M, Ramoni, V, Brucato, A, Airò, P, Bajocchi, G, Bellisai, F, Biasi, D, Blagojevic, J, Canti, V, Caporali, R, Caramaschi, P, Chiarolanza, I, Codullo, V, Cozzi, F, Cuomo, Giovanna, Cutolo, M, De Santis, M, De Vita, S, Di Poi, E, Doria, A, Faggioli, P, Favaro, M, Ferraccioli, G, Ferri, C, Foti, R, Gerosa, A, Gerosa, M, Giacuzzo, S, Gianil, Giuggioli, D, Imazio, M, Iudici, M, Iuliano, A, Leonardi, R, Limonta, M, Lojacono, A, Lubatti, C, Matucci Cerinic, M, Mazzone, A, Meroni, M, Meroni, Pl, Mosca, M, Motta, M, Muscarà, M, Nava, S, Padovan, M, Pagani, G, Paolazzi, G, Peccatori, S, Ravagnani, V, Riccieri, V, Rosato, E, Rovere Querini, P, Salsano, F, Santaniello, A, Scorza, R, Tani, C, Valentini, Gabriele, Valesini, G, Vanoli, M, Vigone, B, Zeni, S, Tincani, A, and on behalf ofthe IMPRESS, Investigators
Abstract: OBJECTIVE: To assess fetal and maternal outcomes in women with systemic sclerosis (SSc). METHODS: Prospectively collected data on 99 women with SSc from 25 Italian centers were analyzed retrospectively. Women with SSc were observed during 109 pregnancies (from 2000 to 2011), and outcomes were compared to those in the general obstetric population (total of 3,939 deliveries). The maternal age at conception was a mean ± SD 31.8 ± 5.3 years, and the median disease duration at conception was 60 months (range 2-193 months). RESULTS: SSc patients, compared to the general obstetric population, had a significantly increased frequency of preterm deliveries (25% versus 12%) and severe preterm deliveries (
Published: 2012

36. Occasional detection of thymic epithelial tumor 4 years after diagnosis of adult onset Still disease A challenging case report and immuno-oncological considerations coming from pertinent literature review

Author: Lococo, Filippo, Bajocchi, G., Caruso, A., Valli, R., Ricchetti, T., Sgarbi, G., Salvarani, C., Lococo F. (ORCID:0000-0002-9383-5554), Lococo, Filippo, Bajocchi, G., Caruso, A., Valli, R., Ricchetti, T., Sgarbi, G., Salvarani, C., and Lococo F. (ORCID:0000-0002-9383-5554)
Abstract: Background: Thymoma is a T cell neoplasm arising from the thymic epithelium that due to its immunological role, frequently undercover derangements of immunity such a tumors and autoimmune diseases. Methods:Herein, we report, to the best of our knowledge, the first description of an association between thymoma and adult onset Still disease (AOSD) in a 47-year-old man. The first one was occasionally detected 4 years later the diagnosis of AOSD, and surgically removed via right lateral thoracotomy. Histology confirmed an encapsulated thymic tumor (type AB sec. WHO-classification). Results: The AOSD was particularly resistant to the therapy, requiring a combination of immunosuppressant followed by anti-IL1R, that was the only steroids-sparing treatment capable to induce and maintain the remission. The differential diagnosis was particularly challenging because of the severe myasthenic-like symptoms that, with normal laboratory tests, were initially misinterpreted as fibromyalgia. The pathogenic link of this association could be a thymus escape of autoreactive T lymphocytes causing autoimmunity. Conclusion: Clinicians should be always include the possibility of a thymoma in the differential diagnosis of an unusual new onset of weakness and normal laboratories data, in particular once autoimmune disease is present in the medical history.
Published: 2016

37. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database

Author: Avouac, Jerome, Walker, Ulrich, Tyndall, Alan, Kahan, André, Matucci Cerinic, Marco, Allanore, Yannick, Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I. Zimmermann, Carreira, P., Novak, S., Czirjak, L., Ramos, F. Oliveira, Jendro, M., Chizzolini, C., Kucharz, E. J., Richter, J., Cozzi, F., Rozman, B., Mallia, C. M., Gabrielli, A., Farge, D., Kiener, H. P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L. M., Morovic Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J. M., Kaltwasser, J. P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J. A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L. Damjanovska, Tikly, M., Nasonov, E. L., Steinbrink, K., Herrick, A., Müller Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R. M., Antivalle, M., Espinosa, I. B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R. M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M. Sinziana, Sunderkötter, C., Jun, J. B., Derk, C., Alhasani, S., Distler, J. H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M. T., Cantatore, F. P., Strauss, G., Von Mülhen, C. A., Pozzi, M. R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F. A., Rom Ivorra, J. A., Krummel Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A. Valderílio, Del Galdo, F., Popa, S., Zenone, T., Machado, X. Ricardo, Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J. Francisco Marques, Bagnato, G. F., Loyo, E., Toloza, S., Li, M., Mohamed, W. Ahmed Abdel Atty, Cobankara, V., Olas, J., Salsano, F., Oksel, F., Tanaseanu, C. M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K. Pasalic, Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., Furst, D. E., VALENTINI, Gabriele, Avouac, Jerome, Walker, Ulrich, Tyndall, Alan, Kahan, André, Matucci Cerinic, Marco, Allanore, Yannick, Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Valentini, Gabriele, Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I. Zimmermann, Carreira, P., Novak, S., Czirjak, L., Ramos, F. Oliveira, Jendro, M., Chizzolini, C., Kucharz, E. J., Richter, J., Cozzi, F., Rozman, B., Mallia, C. M., Gabrielli, A., Farge, D., Kiener, H. P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L. M., Morovic Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J. M., Kaltwasser, J. P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J. A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L. Damjanovska, Tikly, M., Nasonov, E. L., Steinbrink, K., Herrick, A., Müller Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R. M., Antivalle, M., Espinosa, I. B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R. M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M. Sinziana, Sunderkötter, C., Jun, J. B., Derk, C., Alhasani, S., Distler, J. H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M. T., Cantatore, F. P., Strauss, G., Von Mülhen, C. A., Pozzi, M. R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F. A., Rom Ivorra, J. A., Krummel Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A. Valderílio, Del Galdo, F., Popa, S., Zenone, T., Machado, X. Ricardo, Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J. Francisco Marque, Bagnato, G. F., Loyo, E., Toloza, S., Li, M., Mohamed, W. Ahmed Abdel Atty, Cobankara, V., Olas, J., Salsano, F., Oksel, F., Tanaseanu, C. M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K. Pasalic, Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., Furst, D. E., Chizzolini, Carlo, and Westhovens, Rene
Subjects: Male, Systemic disease, Databases, Factual, Cross-sectional study, Joint Diseases/etiology/pathology/physiopathology, Systemic inflammation, Joint involvement, Scleroderma, systemic sclrosis, systemic inflemmetion, joint involvement, Tendons, Systemic sclerosi, Scleroderma, Localized, 0302 clinical medicine, data base, Immunopathology, joint radiography, Immunology and Allergy, Joints/pathology, scleroderma, 030212 general & internal medicine, nuclear magnetic resonance imaging, Range of Motion, Articular, skin and connective tissue diseases, rheumatic disease, ddc:616, interstitial lung disease, Joint contracture, Clinical Trials as Topic, Synovitis, Inflammation/etiology/pathology/physiopathology, integumentary system, article, Tendons/pathology, Middle Aged, musculoskeletal system, cohort analysis, Connective tissue disease, priority journal, Joint, Synoviti, Systemic sclerosis, Female, medicine.symptom, Joint Diseases, Human, musculoskeletal diseases, Adult, medicine.medical_specialty, hand radiography, Immunology, Scleroderma, Localized/etiology/*pathology, Auto-immunity, transplantation and immunotherapy [N4i 4], 03 medical and health sciences, SYSTEMIC SCLEROSIS, JOINT INVOLVEMENT, SYNOVITIS, JOINT CONTRACTURE, TENDON FRICTION RUB, Tendon friction rub, Rheumatology, Internal medicine, medicine, Humans, Health care ethics [NCEBP 5], Tendon, Aged, 030203 arthritis & rheumatology, Cross-Sectional Studie, Inflammation, skin disease, Scleroderma, Systemic, business.industry, echography, medicine.disease, major clinical study, tenosynovitis, Synovitis/etiology/pathology, clinical feature, body regions, Cross-Sectional Studies, Evaluation of complex medical interventions [NCEBP 2], Scleroderma, Systemic/complications/pathology/physiopathology, Joints, disease duration, business, Joint Disease, disease activity
Abstract: Objective.To determine the prevalence of and independent factors associated with joint involvement in a large population of patients with systemic sclerosis (SSc).Methods.This study was cross-sectional, based on data collected on patients included in the European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) registry. We queried this database to extract data regarding global evaluation of patients with SSc and the presence of any clinical articular involvement: synovitis (tender and swollen joints), tendon friction rubs (rubbing sensation detected as the tendon was moved), and joint contracture (stiffness of the joints that decreased their range of motion). Overall joint involvement was defined by the occurrence of synovitis and/or joint contracture and/or tendon friction rubs.Results.We recruited 7286 patients with SSc; their mean age was 56 ± 14 years, disease duration 10 ± 9 years, and 4210 (58%) had a limited cutaneous disease subset. Frequencies of synovitis, tendon friction rubs, and joint contractures were 16%, 11%, and 31%, respectively. Synovitis, tendon friction rubs, and joint contracture were more prevalent in patients with the diffuse cutaneous subset and were associated together and with severe vascular, muscular, renal, and interstitial lung involvement. Moreover, synovitis had the highest strength of association with elevated acute-phase reactants taken as the dependent variable.Conclusion.Our results highlight the striking level of articular involvement in SSc, as evaluated by systematic examination in a large cohort of patients with SSc. Our data also show that synovitis, joint contracture, and tendon friction rubs are associated with a more severe disease and with systemic inflammation.
Published: 2010

38. Evidence of a selective nociceptive impairment in systemic sclerosis

Author: Bajocchi, G., Terlizzi, R., Zanigni, S., GIORGIO BARLETTA, Grimaldi, D., Pierangeli, G., Cortelli, P., Bajocchi G., Terlizzi R., Zanigni S., Barletta G., Grimaldi D., Pierangeli G., and Cortelli P.
Published: 2009

39. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials And Research group database

Author: Walker U. A., Tyndall A., Czirják L, Denton C. ., Farge Bancel D., Kowal Bielecka O., Müller Ladner U., Bocelli Tyndall C., Matucci Cerinic M., Riemekasten G. ., Brückner C. ., Airó P. ., Scarsi M. ., Scorza R. ., Beretta L. ., Cozzi F. ., Tiso F., Vonk Mc M. C., Hoogen Van Den, Fhj F. H. J., Wigley Fm F. M., Hummers L. ., Nevskaya T. ., Ananieva L. ., Miniati I. ., Tartaglia N. ., Lomater C. ., Balbir Gurman A. ., Braun Moscovici Y. ., Bambara Lm L. M., Caramaschi P. ., Ruocco L. ., Krieg T. ., Hunzelmann N. ., Varjú C. ., Carriera Pe P. E., Joven B. ., Iannone F. ., Lapadula G. ., Kahan A. ., Allanore Y. ., Gabrielli A. ., Imperatore M. ., Scheja A. ., Wollheim F. ., Damjanov N. ., Ostojic P. ., Saar P. ., Tarner Ih I. H., Kötter I. ., Bombardieri S. ., Bazzichi L. ., Papa Del N. ., Comina Dp D. P., Monaco Lo A. ., Corte La R. ., Hachulla E. ., Launay D. ., Distler O. ., Ciurea A. ., Sierakowski S. ., Mitchell H. ., Silver Rm R. M., Krasowska D. ., Michalska Jakubus M. ., Tikly M. ., Aboo N. ., Worm M. ., Klaus P. ., Rovenský J. ., Lukáčová O. ., Rozman B. ., Sipek A. ., Clemente Coelho P. ., Shoenfeld Y. ., Langewitch P. ., José Da Silva Ap A. P., Salvador Mj M. J., Kuhn A. ., Erdmann G. ., Bečvář R. ., Friedl E. ., Graninger W. ., Riccieri V. ., Caporali R. ., Montecucco C. ., Vlachoyiannopoulos P. ., Distler M. ., Reich K. ., Majdan M. ., Wielosz E. ., Rednic S. ., Laar Van Jm J. M., Heitmann S. ., Bruckner A. ., Himsel A. ., Riemann J. ., Meyringer R. ., Müller A. ., Martinovic D. ., Radic M. ., Sticherling M. ., Szekanecz Z. ., Szücs G. ., Giacomelli R. ., Marrelli A. ., Stamenkovic B. ., Stankovic A. ., Aringer M. ., Smolen Js J. S., Kucharz Ej E. J., Kotulska At A. T., Jablonska S. ., Blasczik M. ., Jun J. B. J. B., Mallia C. ., Coleiro B. ., Santamaria Vo V. O., Hinrichs R. ., Nielsen H. ., Cossutta R. ., Ionescu R. ., Opris D. ., Steinbrink K. ., Grundt B. ., Bajocchi G. ., Jiří Š. ., Lefebvre Pgdlp P. G. D. L. P., Mendoza ZeaAc A. C., Ribi C. ., Chizzolini C. ., Wisłowska M. ., Novak S. ., Indiveri F. ., Jacobsen S. ., Frandsen Pb P. B., Gorska Iz I. Z., Gran Tore J. ., Midtvedt Ø. ., Ramos Fo F. O., Rajcevska Ld L. D., Bozinovski G. ., Schöffel D. ., Sunderkötter C. ., Böhm M. ., Morović Vergles J. ., Čulo M. I. M. I., Cutolo M. ., Sulli A. ., Derk Ct C. T., Jimenez Sa S. A., Siakka P. ., Søndergaard K. ., Stengaard Pedersen K. ., Cabane J. ., Tiev Kp K. P., Mihai C. ., Sfrent Cornateanu R. ., Jendro M. ., Tuvik P. ., Antivalle M. ., Randisi G. ., Seidel M. ., Clarenbach R. ., Simsek I. ., Dinc A. ., Inanc M. ., Capraru Ms M. S., Capraru D. ., Bañegil I. ., Richter J. ., Alhasani S. ., Földvari I. ., Pinto S. ., Brandão F. ., VALENTINI, Gabriele, Walker, U. A., Tyndall, A., Czirják, L, Denton, C. ., Farge Bancel, D., Kowal Bielecka, O., Müller Ladner, U., Bocelli Tyndall, C., Matucci Cerinic, M., Riemekasten, G. ., Brückner, C. ., Airó, P. ., Scarsi, M. ., Scorza, R. ., Beretta, L. ., Cozzi, F. ., Tiso, F., Vonk Mc, M. C., Hoogen Van, Den, Fhj, F. H. J., Wigley Fm, F. M., Hummers, L. ., Nevskaya, T. ., Ananieva, L. ., Miniati, I. ., Tartaglia, N. ., Lomater, C. ., Balbir Gurman, A. ., Braun Moscovici, Y. ., Bambara Lm, L. M., Caramaschi, P. ., Valentini, Gabriele, Ruocco, L. ., Krieg, T. ., Hunzelmann, N. ., Varjú, C. ., Carriera Pe, P. E., Joven, B. ., Iannone, F. ., Lapadula, G. ., Kahan, A. ., Allanore, Y. ., Gabrielli, A. ., Imperatore, M. ., Scheja, A. ., Wollheim, F. ., Damjanov, N. ., Ostojic, P. ., Saar, P. ., Tarner Ih, I. H., Kötter, I. ., Bombardieri, S. ., Bazzichi, L. ., Papa Del, N. ., Comina Dp, D. P., Monaco Lo, A. ., Corte La, R. ., Hachulla, E. ., Launay, D. ., Distler, O. ., Ciurea, A. ., Sierakowski, S. ., Mitchell, H. ., Silver Rm, R. M., Krasowska, D. ., Michalska Jakubus, M. ., Tikly, M. ., Aboo, N. ., Worm, M. ., Klaus, P. ., Rovenský, J. ., Lukáčová, O. ., Rozman, B. ., Sipek, A. ., Clemente Coelho, P. ., Shoenfeld, Y. ., Langewitch, P. ., José Da Silva Ap, A. P., Salvador Mj, M. J., Kuhn, A. ., Erdmann, G. ., Bečvář, R. ., Friedl, E. ., Graninger, W. ., Riccieri, V. ., Caporali, R. ., Montecucco, C. ., Vlachoyiannopoulos, P. ., Distler, M. ., Reich, K. ., Majdan, M. ., Wielosz, E. ., Rednic, S. ., Laar Van Jm, J. M., Heitmann, S. ., Bruckner, A. ., Himsel, A. ., Riemann, J. ., Meyringer, R. ., Müller, A. ., Martinovic, D. ., Radic, M. ., Sticherling, M. ., Szekanecz, Z. ., Szücs, G. ., Giacomelli, R. ., Marrelli, A. ., Stamenkovic, B. ., Stankovic, A. ., Aringer, M. ., Smolen Js, J. S., Kucharz Ej, E. J., Kotulska At, A. T., Jablonska, S. ., Blasczik, M. ., Jun, J. B. J. B., Mallia, C. ., Coleiro, B. ., Santamaria Vo, V. O., Hinrichs, R. ., Nielsen, H. ., Cossutta, R. ., Ionescu, R. ., Opris, D. ., Steinbrink, K. ., Grundt, B. ., Bajocchi, G. ., Jiří, Š. ., Lefebvre Pgdlp, P. G. D. L. P., Mendoza ZeaAc, A. C., Ribi, C. ., Chizzolini, C. ., Wisłowska, M. ., Novak, S. ., Indiveri, F. ., Jacobsen, S. ., Frandsen Pb, P. B., Gorska Iz, I. Z., Gran Tore, J. ., Midtvedt, Ø. ., Ramos Fo, F. O., Rajcevska Ld, L. D., Bozinovski, G. ., Schöffel, D. ., Sunderkötter, C. ., Böhm, M. ., Morović Vergles, J. ., Čulo, M. I. M. I., Cutolo, M. ., Sulli, A. ., Derk Ct, C. T., Jimenez Sa, S. A., Siakka, P. ., Søndergaard, K. ., Stengaard Pedersen, K. ., Cabane, J. ., Tiev Kp, K. P., Mihai, C. ., Sfrent Cornateanu, R. ., Jendro, M. ., Tuvik, P. ., Antivalle, M. ., Randisi, G. ., Seidel, M. ., Clarenbach, R. ., Simsek, I. ., Dinc, A. ., Inanc, M. ., Capraru Ms, M. S., Capraru, D. ., Bañegil, I. ., Richter, J. ., Alhasani, S. ., Földvari, I. ., Pinto, S. ., Brandão, F. ., Ribi, Camillo, and Chizzolini, Carlo
Subjects: Male, Systemic disease, Databases, Factual, Cross-sectional study, Scleroderma, Immunopathology, Immunology and Allergy, Age of Onset, skin and connective tissue diseases, ddc:616, integumentary system, Nuclear Proteins, Orvostudományok, Middle Aged, Connective tissue disease, Extended Report, Raynaud Disease/etiology/immunology, DNA Topoisomerases, Type I, Nuclear Proteins/immunology, Female, Adult, medicine.medical_specialty, Scleroderma, Diffuse/complications/immunology, Immunology, Klinikai orvostudományok, Scleroderma, Systemic/complications/immunology, Risk Assessment, General Biochemistry, Genetics and Molecular Biology, systemic sclerosis, EULAR, risk assessment, Age Distribution, Sex Factors, Rheumatology, Scleroderma, Limited, medicine, Humans, Risk factor, Aged, Autoantibodies, Scleroderma, Systemic, business.industry, Autoantibody, Raynaud Disease, medicine.disease, Dermatology, Cross-Sectional Studies, Scleroderma, Diffuse, Scleroderma, Limited/complications/immunology, Age of onset, business, Autoantibodies/blood
Abstract: Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (lcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and Methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the ACR diagnostic criteria in participating centres. We aimed to characterize demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with lcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were female. On multivariate analysis, scleroderma subsets (dcSSc vs. lcSSc), antibody status and age at onset of Raynaud’s phenomenon, but not gender were independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis appeared more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and lcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction appeared superseded by an antibody based classification in predicting some scleroderma complications. The EUSTAR MEDS data base facilitates the analysis of clinical patterns in SSc and contributes to the standardised assessment and monitoring of SSc internationally.
Published: 2007

40. DUO Registry Group. Functional impairment of systemic scleroderma patients with digital ulcerations: results from the DUO Registry

Author: Guillevin, L, Hunsche, E, Denton, Cp, Krieg, T, Schwierin, B, Rosenberg, D, Matucci Cerinic, M, DUO Registry Group Collaborators Raffier, B, Hirschi, M, Trautinger, F, Schmidt, P, Stetter, M, Hundstorfer, M, Reinhart, V, Monshi, B, Pirkhammer, D, Richter, L, Hamberger, N, Metz, S, Feldmann, R, Semmelweis, K, Lackner, K, Tomi, N, Kolle, H, Hafner, F, Brodmann, M, Kuen Spiegel, M, Minmair, G, Heil, Pm, Broil, H, Holzer, G, Illmer, X, Rintelen, B, Sautner, J, Takacs, M, Thun, M, Zemanova, I, Soukup, T, Smrzova, A, Bohmova, J, Prochazkova, L, Nemec, P, Fojtik, Z, Suchy, D, Becvar, R, Olsen, Ab, Sondergaard, Kh, Luosu jarvi, R, Vidqvist, Kl, Madaule, S, Beneton, N, Maillard, H, Charlanne, H, Granelbrocard, F, Hachulla, E, Hatron, Py, Jourdain, N, Lambert, M, Launay, D, Morell, S, Woijtasik, G, Skowron, F, Zenone, T, Dadban, A, Lok, C, Ferrandiz, D, Magybertrand, N, Moiton, Mp, Taieb, A, Balquiere, S, Belin, E, Droitcourt, C, Julien, S, Prey, S, Boulon, C, Constans, J, Doutre, Ms, Kostrzwewa, E, Richez, C, Greco, M, Misery, L, Sassolas, B, Collet, E, Berthier, S, Leguy Seguin, V, Imbert, B, Carpentier, P, Blaise, S, Couraud, A, Doeffel Hantz, V, Spars, A, Bezanahary, H, Boussely, N, Dumonteil, S, Fauchais, Al, Goudran, G, Loustaud Ratti, V, Manea, P, Vidal, E, Coppere, B, Desmursclavel, H, Girard Madoux MH, Hot, A, Ninet, J, Granel, B, Cohen, Jd, Keynote, A, Khau van Kien, A, Le Quellec, A, Riviere, S, Rullier, P, Bessis, D, Farcas, C, Bravetti, V, Moline, T, Wahl, D, Zuily, S, Granel Brocard, F, Agard, C, Durant, C, Fuzibet, Jg, Queyrel, V, Berezne, A, Mouthon, L, Cabane, J, Tiev, K, Toledano, C, Lazareth, I, Michon Pasturel, U, Priollet, P, Reguiai, Z, Cazaletslacoste, C, Jego, P, Letremy, A, Perlat, A, Duval Modeste AB, Chatelus, E, Chiffot, H, Sibillia, J, Sordet, C, Adoue, D, Couret, B, Moulis, G, Pugnet, G, Sailler, L, Diot, E, Gaches, F, Farge, D, Keshtmand, H, Frances, C, von Elling, A, Bora, D, Ebel, J, Ahmadi Simab, K, Klein, E, Hahn, K, Schulze, K, Rasche, C, Riemekasten, G, Lee, Hh, Deuschle, K, Mattat, K, Becker, M, Worm, M, Mensing, C, Klings, D, Mensing, H, Messall, J, Zuper, R, Eilbacher, P, Saar, P, Kaufmann, P, Hallermann, C, Schmidt, K, Wahn, H, Schildt, K, Schuart, T, Kaczmarczyk, A, Kellner, C, von Oelhafen, J, Baron von Bildering, P, Kunze, S, Kleiner, Hj, Alsheimer, B, Schuetz, N, Miirker Hermann, E, Gottl, Kh, Weiss, E, Reischel, N, Kern, S, Goettl, Kh, Goetheuniversitiitsklinikum, Jw, Himsel, A, Henkemeier, U, Schwarting, A, Hazenbiller, A, Nichelmann, V, Rumbaur, C, Boesenberg, I, Schmeiser, T, Mueller Ladner, U, Unholzer, A, Starz, H, Welzel, J, Plaumann, K, Stoeckl, F, Sperling, S, Podda, M, Wagner, N, Rapprich, H, Niedermeier, A, Messer, G, Sardy, M, Bekou, V, Dill MUller, D, Wlodarz, M, Belloni, B, Huettig, B, Ziai, M, Hein, R, Kneitz, C, Federow, I, Schneider, K, Semmler, M, Hapke, S, Metzler, C, Stein, T, Enderlein, M, Kayser, M, Werthmann, M, Guenther, Cu, Neul, S, Hellmich, B, Loeffler, C, Pflugfelder, J, Karaenke, P, Mueglich, C, Tony, Hp, Marina, P, Popp, M, Mittag, M, Baumann, C, Scheib, Eg, Brand, H, Wilhelm, Hu, Bohm, J, Dyballa, J, Boehm, J, Taggeselle, J, Luthke, K, Wuerzburg, I, Niefanger, K, Mayer, L, Drabek, J, Harmuth, W, Dietl, S, Moritz, D, Gause, A, Gaubitz, M, Hallecker, A, Krupp, E, Rumpel, H, Moosig, F, Frey, P, Kahl, S, Linke, M, Merk, B, Bloching, Hh, Ochs, W, Kurthen, R, Eiden, E, Guertler, I, Aries, Pm, Kirchberg, S, Jahnke, K, Mettler, S, Toeller, S, Zwenger, S, Langer, He, Deininger, F, Hartmann, F, Neeck, G, Neek, G, Wernitzsch, H, Meier, L, Herr, U, Meier, U, Aaig, W, Bruckner, L, Sheikh, N, Wollenhaupt, J, Krog, B, Wollersdorfer, E, Hall, R, Diehm, C, Tiggers, C, Peters, J, Kirschke, J, Schroeder, Jo, Zeuner, R, Uhlig, S, Barth, S, Huegel, R, Glaeser, R, Schaefer, C, Monshausen, M, Mengden, T, Funkert, A, Blank, N, Lupaschko, S, Voss, B, Megahed, M, Sadeghlar, F, Seidel, M, Wasmuth, Jc, Kreuter, A, Vosswinkel, J, Pfoehler, C, Gerber, A, Haust, M, Hoff, Np, Mota, R, Akanay Diesel, S, Homey, B, Katzemich, A, Erfurt Berge, C, Sticherling, M, Beyer, C, Distler, J, Mitchell, A, Freundlieb, C, Rushentsova, U, Hermanns, G, Blaschke, S, Fiene, M, Wessel, C, Norgauer, J, Rabe, B, Schuster, J, Scholz, J, Kremer, K, Robakidze Torbahn, M, Moinzadeh, P, Dohse, A, Muhlack, A, Schultz, L, Schult, S, Frambach, Y, Kruse, S, Kettenbach, A, Fell, I, Schweda, K, Steinbrink, K, Podobinska, M, Fieri beck, G, Schanz, S, Pfeiffer, C, Hassel, R, Herrgott, I, Sunderkoetter, C, Guenzel, J, Athanassiou, P, Dimitroulas, T, Settas, L, Kritikos, I, Tsifetaki, N, Garyfallos, A, Vasilopoulos, D, Boura, P, Kamali, S, Aslanidis, S, Vlachoyannopoulos, P, Galanopoulo, V, Sakkas, L, Koutroubas, A, Elezoglou, T, Galanopoulos, N, Grier, A, Murray, M, O'Rourke, M, Del Papa, N, Maglione, W, Zeni, S, Foti, R, Benenati, A, De Vita, S, Ferraccioli, G, Grassi, W, de Angeli, R, Pomponio, G, Mussi, A, Colonna, L, Airo, P, Zingarelli, S, Scorza, R, Serverino, A, Puppo, F, Negrini, S, Roma, I, Salsano, F, Triolo, G, Mazzuca, S, Carignola, R, Gatti, S, Lunardi, G, Riccieri, V, Salvarani, C, Bajocchi, G, Varcasia, G, Marasini, B, Belloll, L, de Luca, R, Stisi, S, Bellissimo, S, Fusaro, E, Pellerito, R, Cozzi, F, Rizzo, M, Bartoluzzi, A, Trotta, F, Cantatore, F, Corrado, A, Ferri, Claudio, Colaci, M, Malavolta, N, Mule, R, Galeazzi, M, Lapadula, G, Mathieu, A, Vacca, A, Giacomelli, Roberto, Cipriani, Paola, Montecucco, Cm, Codullo, V, Bucci, R, Battaglia, E, Valentini, G, Cuomo, G, Terlizzi, N, Serafino, L, Reumatologia, Uo, Bombardieri, S, Della Rossa, A, Doveri, M, Perricone, R, de Mattia, M, Pallotta, S, Groenendael, Jh, Seys, P, Goekoop, Rj, Han, Kh, Wlarvens, M, Bonte Mineur, F, de Bois MH, de Beus WM, van Zeben, D, Vonk, M, Knaapen, Hk, Smit, A, Bootsma, H, Ton, E, Voskuyl, A, Dutmer, Ea, Stalk, Jn, Madland, Tm, Seip, M, Hoffmann Vold AM, Bitter, H, Stocklund Thomsen, R, Resende, C, Ponte, C, Martinho, S, Silva, F, Ferreira, P, Grilo, A, Riso, N, Santos, C, Camara, I, Costa, J, Alves, J, Oliveira, S, Almeida, I, Silva, I, Cordeiro, A, Coelho, P, Lukac, J, Dolnicar, As, Espinosa, G, Mejia, Jc, Ramos, M, Plasin Rodriguez MA, Mera, A, Blanco, Js, Diaz, Jj, Losada, L, Perez, E, Maneiro, Jr, Caamano, M, Fermindez, S, Insua, Sa, Barbado, J, Fonseca, Em, Nufio, Fj, Castellvi, I, Garcia de Ia Pena, P, Bellido, D, Paulino, M, Garcia, Pv, Salas, V, Minguez, Md, Sanchez, Ma, Urrego, C, Martin, I, Rueda, A, Calvo, J, Ripoll, Mm, Torres, Mc, Corteguera, M, Maceiras, F, Cruz, J, Mosquera, Ja, Gomez, R, Area, B, Carrio, I, Rubio, M, Castellvi Barranco, I, Santos, P, Simeon, Cp, Fonollosa, V, Egurbide, Mv, Garcia de Vicuna, R, Vicente, E, Villaverde, V, Fernandez, C, Garcia, E, Uson, J, Miguelez, R, Callejas, Jl, Ortego, N, Roman, J, Alegre Sancho JJ, Robles, A, Rios, Jj, Bonilla, Mg, Sanchez Andrade, A, Vazquez, Tr, Miranda, Ja, Saez, L, Zea, A, De la Puente, C, Martinez, Fg, Aguirre, Ma, Collado, P, Cruz, A, Crespo, M, Sanchez Roman, J, Castillo, Mj, Garcia, Am, Muniz, G, Hedin, Pj, Stahl, C, Bracin, T, Nordin, A, Albertsson, K, Rydvald, Y, Thorsson, C, Hermansson, E, Maurer, B, Verner, D, Schmidt Bosshard, R, Hall, F, Murphy, K, Lamb, J, Anderson, M, Moots, R, Buch, M, Bissell, L, Madhok, R, Hampson, R, D'Cruz, D, Choong, Lm, Gordon, P, Dobson, J, Salerno, R, Nisar, M, Williams, C, Wilcox, L, Denton, C, Ochiel, R, Ngcozana, T, Parker, L, Vincent, R, Mchugh, N, Cole, S, Brown, S, James, J, Herrick, A, Manning, J, Moore, T, Faizal, A, Skyes, H, Smythe, A, and Hamilton, A.
Published: 2013

41. Systemic sclerosis lung disease in limited (C.R.E.S.T.) and diffuse subset

Author: Bajocchi, G., Carbonelli, C., Zucchi, L., and Salvarani, C.
Subjects: NSIP, UIP, Interstitial lung disease, Sistemic sclerosis
Published: 2013

42. Antisynthetase syndrome

Author: Carbonelli, C., Bajocchi, G., Salvarani, C., and Zucchi, L.
Subjects: Antisynthetase syndrome, ARDS, Diffuse interstitial lung disease, Idiopathic inflammatory myopathy
Published: 2012

43. Brief Report: Successful pregnancies but a higher riskof preterm births in patients with systemic sclerosis: An Italian multicenterstudy

Author: Taraborelli, M, Ramoni, V, Brucato, A, Airò, P, Bajocchi, G, Bellisai, F, Biasi, D, Blagojevic, J, Canti, V, Caporali, R, Caramaschi, P, Chiarolanza, I, Codullo, V, Cozzi, Franco, Cuomo, G, Cutolo, M, De Santis, M, De Vita, S, Di Poi, E, Doria, Andrea, Faggioli, P, Favaro, M, Ferraccioli, G, Ferri, C, Foti, R, Gerosa, A, Gerosa, M, Giacuzzo, S, Giani, L, Giuggioli, D, Imazio, M, Iudici, M, Iuliano, A, Leonardi, R, Limonta, M, Lojacono, A, Lubatti, C, Matucci Cerinic, M, Mazzone, A, Meroni, M, Meroni, Pl, Mosca, M, Motta, M, Muscarà, M, Nava, S, Padovan, M, Pagani, G, Paolazzi, G, Peccatori, S, Ravagnani, V, Riccieri, V, Rosato, E, Rovere Querini, P, Salsano, F, Santaniello, A, Scorza, R, Tani, C, Valentini, G, Valesini, G, Vanoli, M, Vigone, B, Zeni, S, and Tincani, A.
Published: 2012

44. Successful pregnancies but a higher risk of preterm births in systemic sclerosis: An Italian multicentric study

Author: Taraborelli, M, Ramoni, V, Brucato, A, Airò, P, Bajocchi, G, Bellisai, F, Biasi, D, Blagojevic, J, Canti, V, Caporali, R, Caramaschi, P, Chiarolanza, I, Codullo, V, and Cozzi, Franco
Published: 2011

45. FRI0477 Isolated Arthritis Revealing an Underlying Anti-Synthetase Syndrome: Results from a Multicentre International Study

Author: Cavagna, L., primary, Govoni, M., additional, Lopez Longo, F.J., additional, Airò, P., additional, Neri, R., additional, Sifuentes Giraldo, W.A., additional, Iannone, F., additional, Nuno, L., additional, Montecucco, C., additional, Caporali, R., additional, Furini, F., additional, Foschi, V., additional, Franceschini, F., additional, Cavazzana, I., additional, Quartuccio, L., additional, Bartoloni Bocci, E., additional, Giannini, M., additional, Sciré, C., additional, Fusaro, E., additional, Parisi, S., additional, Paolazzi, G., additional, Barausse, G., additional, Selmi, C., additional, Bachiller Corral, J., additional, Bravi, E., additional, Bajocchi, G., additional, Pellerito, R., additional, Russo, A., additional, Barsotti, S., additional, Pina Murcia, T., additional, Castaneda, S., additional, Ortego-Centeno, N., additional, Schwarting, A., additional, Specker, C., additional, Saketkoo, L.A., additional, Weinmann-Menke, J., additional, Triantafyllias, K., additional, and Gonzalez-Gay, M.-A., additional
Published: 2015
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46. Characteristics of joint involvement and relationship with systemic inflammation in systemic sclerosis: result from the EULAR scleroderma trial and research (EUSTAR) database

Author: Avouac, J, Walker, U, Tyndall, A, Kahan, A, Matucci Cerinic, M, Allanore, Y, Eustar, Miniati, I, Muller, A, Iannone, F, Distler, O, Becvar, R, Sierakowsky, S, Kowal Bielecka, O, Coelho, P, Cabane, J, Cutolo, M, Shoenfeld, Y, Valentini, G, Rovensky, J, Riemekasten, G, Vlachoyiannopoulos, P, Caporali, R, Jiri, S, Inanc, M, Zimmermann Gorska, I, Carreira, P, Novak, S, Czirjak, L, Oliveira Ramos, F, Jendro, M, Chizzolini, C, Kucharz, Ej, Richter, J, Cozzi, F, Rozman, B, Mallia, Cm, Gabrielli, A, Farge, D, Kiener, Hp, Schöffel, D, Airo, P, Wollheim, F, Martinovic, D, Trotta, F, Jablonska, S, Reich, K, Bombardieri, S, Siakka, P, Pellerito, R, Bambara, Lm, Morovic Vergles, J, Denton, C, Hinrichs, R, Van den Hoogen, F, Damjanov, N, Kötter, I, Ortiz, V, Heitmann, S, Krasowska, D, Seidel, M, Hasler, P, Van Laar JM, Kaltwasser, Jp, Foeldvari, I, Juan Mas, A, Bajocchi, G, Wislowska, M, Pereira Da Silva JA, Jacobsen, S, Worm, M, Graniger, W, Kuhn, A, Stankovic, A, Cossutta, R, Majdan, M, Damjanovska Rajcevska, L, Tikly, M, Nasonov, El, Steinbrink, K, Herrick, A, Müller Ladner, U, Dinc, A, Scorza, R, Sondergaard, K, Indiveri, F, Nielsen, H, Szekanecz, Z, Silver, Rm, Antivalle, M, Espinosa, Ib, García de la Pena Lefebvre, P, Midtvedt, O, Launay, D, Valesini, F, Tuvik, P, Ionescu, Rm, Del Papa, N, Pinto, S, Wigley, F, Mihai, C, Sinziana Capranu, M, Sunderkötter, C, Jun, Jb, Alhasani, S, Distler, Jh, Ton, E, Soukup, T, Seibold, J, Zeni, S, Nash, P, Mouthon, L, De Keyser, F, Duruöz, Mt, Cantatore, Fp, Strauss, G, von Mülhen CA, Pozzi, Mr, Eyerich, K, Szechinski, J, Keiserman, M, Houssiau, Fa, Román Ivorra JA, Krummel Lorenz, B, Aringer, M, Westhovens, R, Bellisai, F, Mayer, M, Stoeckl, F, Uprus, M, Volpe, A, Buslau, M, Yavuz, S, Granel, B, Valderílio Feijó, A, Del Galdo, F, Popa, S, Zenone, T, Ricardo Machado, X, Pileckyte, M, Stebbings, S, Mathieu, A, Tulli, A, Tourinho, T, Souza, R, Acayaba de Toledo, R, Stamp, L, Solanki, K, Veale, D, Francisco Marques Neto, J, Bagnato, Gf, Loyo, E, Toloza, S, Li, M, Ahmed Abdel Atty Mohamed, W, Cobankara, V, Olas, J, Salsano, F, Oksel, F, Tanaseanu, Cm, Foti, R, Ancuta, C, Vonk, M, Caramaschi, Paola, Beretta, L, Balbir, A, Chiàla, A, Pasalic Simic, K, Ghio, M, Stamenkovic, B, Rednic, S, Host, N, Hachulla, E, and Furst, D. E.
Subjects: joint involvement, Systemic sclerosis, synovitis
Published: 2010

47. Characteristics of joint involvement and relationships with systemic inflammation in systemic sclerosis: Results from the EULAR Scleroderma Trial and Research Group (EUSTAR) database

Author: Avouac, J., Walker, U., Tyndall, A., Kahan, A., Matucci-Cerinic, M., Allanore, Y., Miniati, I., Müller, A., Iannone, F., Giacomelli, R., Distler, O., Becvar, R., Sierakowsky, S., Kowal-Bielecka, O., Coelho, P., Cabane, J., Cutolo, M., Shoenfeld, Y., Valentini, G., Rovensky, J., Riemekasten, G., Nicoara, I., Vlachoyiannopoulos, P., Caporali, R., Jiri, S., Inanc, M., Gorska, I.Z., Carreira, P., Novak, S., Czirjak, L., Ramos, F.O., Jendro, M., Chizzolini, C., Kucharz, E.J., Richter, J., Cozzi, F., Rozman, B., Mallia, C.M., Gabrielli, A., Farge, D., Kiener, H.P., Schöffel, D., Sticherling, M., Airo, P., Wollheim, F., Martinovic, D., Trotta, F., Hunzelmann, N., Jablonska, S., Reich, K., Bombardieri, S., Siakka, P., Pellerito, R., Bambara, L.M., Morovic-Vergles, J., Denton, C., Hinrichs, R., Van Den Hoogen, F., Damjanov, N., Kötter, I., Ortiz, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Van Laar, J.M., Kaltwasser, J.P., Foeldvari, I., Juan Mas, A., Bajocchi, G., Wislowska, M., Pereira Da Silva, J.A., Jacobsen, S., Worm, M., Graniger, W., Kuhn, A., Stankovic, A., Cossutta, R., Majdan, M., Rajcevska, L.D., Tikly, M., Nasonov, E.L., Steinbrink, K., Herrick, A., Müller-Ladner, U., Dinc, A., Scorza, R., Sondergaard, K., Indiveri, F., Nielsen, H., Szekanecz, Z., Silver, R.M., Antivalle, M., Espinosa, I.B., García De La Pena Lefebvre, P., Midtvedt, O., Launay, D., Valesini, F., Tuvik, P., Ionescu, R.M., Del Papa, N., Pinto, S., Wigley, F., Mihai, C., Capranu, M.S., Sunderkötter, C., Jun, J.B., Derk, C., Alhasani, S., Distler, J.H., Ton, E., Soukup, T., Seibold, J., Zeni, S., Nash, P., Mouthon, L., De Keyser, F., Duruöz, M.T., Cantatore, F.P., Strauss, G., Von Mülhen, C.A., Pozzi, M.R., Eyerich, K., Szechinski, J., Keiserman, M., Houssiau, F.A., Rom-Ivorra, J.A., Krummel-Lorenz, B., Aringer, M., Westhovens, R., Bellisai, F., Mayer, M., Stoeckl, F., Üprus, M., Volpe, A., Buslau, M., Yavuz, S., Granel, B., Feijó, A.V., Del Galdo, F., Popa, S., Zenone, T., Machado, X.R., Pileckyte, M., Stebbings, S., Mathieu, A., Tulli, A., Tourinho, T., Souza, R., Acayaba De Toledo, R., Stamp, L., Solanki, K., Veale, D., Neto, J.F.M., Bagnato, G.F., Loyo, E., Toloza, S., Li, M., Mohamed, W.A.A.A., Çobankara, Veli, Olas, J., Salsano, F., Oksel, F., Tanaseanu, C.M., Foti, R., Ancuta, C., Vonk, M., Caramashi, P., Beretta, L., Balbir, A., Shine, B., Chiàla, A., Simic, K.P., Ghio, M., Stamenkovic, B., Rednic, S., Host, N., Hachulla, E., and Furst, D.E.
Subjects: musculoskeletal diseases, Adult, Male, Databases, Factual, systemic sclerosis, joint contracture, hand radiography, Joint involvement, Tendons, Scleroderma, Localized, data base, joint radiography, Humans, scleroderma, human, nuclear magnetic resonance imaging, Range of Motion, Articular, rheumatic disease, Aged, interstitial lung disease, Inflammation, skin disease, Clinical Trials as Topic, Synovitis, Scleroderma, Systemic, integumentary system, article, echography, Middle Aged, musculoskeletal system, cohort analysis, major clinical study, tenosynovitis, clinical feature, body regions, female, Cross-Sectional Studies, priority journal, Tendon friction rub, Joints, disease duration, Joint Diseases, disease activity
Abstract: Objective. To determine the prevalence of and independent factors associated with joint involvement in a large population of patients with systemic sclerosis (SSc). Methods. This study was cross-sectional, based on data collected on patients included in the European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) registry. We queried this database to extract data regarding global evaluation of patients with SSc and the presence of any clinical articular involvement: synovitis (tender and swollen joints), tendon friction rubs (rubbing sensation detected as the tendon was moved), and joint contracture (stiffness of the joints that decreased their range of motion). Overall joint involvement was defined by the occurrence of synovitis and/or joint contracture and/or tendon friction rubs. Results. We recruited 7286 patients with SSc; their mean age was 56 ± 14 years, disease duration 10 ± 9 years, and 4210 (58%) had a limited cutaneous disease subset. Frequencies of synovitis, tendon friction rubs, and joint contractures were 16%, 11%, and 31%, respectively. Synovitis, tendon friction rubs, and joint contracture were more prevalent in patients with the diffuse cutaneous subset and were associated together and with severe vascular, muscular, renal, and interstitial lung involvement. Moreover, synovitis had the highest strength of association with elevated acute-phase reactants taken as the dependent variable. Conclusion. Our results highlight the striking level of articular involvement in SSc, as evaluated by systematic examination in a large cohort of patients with SSc. Our data also show that synovitis, joint contracture, and tendon friction rubs are associated with amore severe disease and with systemic inflammation. The Journal of Rheumatology Copyright © 2010. All rights reserved.
Published: 2010

48. 1T magnetic resonance imaging in the diagnosis of giant cell arteritis: comparison with ultrasonography and physical examination of temporal arteries

Author: Ghinoi, A., Zuccoli, G., Nicolini, A., Pipitone, N., Macchioni, L., Bajocchi, G. L., Nicoli, F., Silingardi, M., Mariagrazia Catanoso, Boiardi, L., and Salvarani, C.
Subjects: Aged, Aged, 80 and over, Biopsy, Cohort Studies, Female, Giant Cell Arteritis, Humans, Male, Sensitivity and Specificity, Temporal Arteries, Diffusion Magnetic Resonance Imaging, 80 and over, Ultrasonography
Abstract: To assess the usefulness of 1T magnetic resonance imaging (MRI) of temporal arteries and to compare 1T MRI with duplex ultrasonography (US) and physical examination of temporal arteries for the diagnosis of giant cell arteritis (GCA) in patients with suspected GCA.The superficial temporal arteries of 20 consecutive patients with a suspected diagnosis of GCA were examined using a 1T MRI scanner. Fat-saturated multislice T1-weighted spin-echo images were acquired perpendicularly to the orientation of the vessel. In all cases, MRI results were compared to US and temporal artery examination findings. Temporal artery biopsies were performed in all patients.Mural contrast enhancement of the temporal arteries on MRI had a sensitivity of only 33.3% and a specificity of 87.5% for the diagnosis of biopsy-proven GCA. Compared with the diagnosis of GCA by the American College of Rheumatology criteria, MRI had a sensitivity and specificity of 27.2% and 88.9%, respectively. Temporal artery abnormalities on physical examination and the presence of a hypoechoic halo on US had a higher sensitivity (66.7% and 77.7%, respectively) and a higher specificity (100% for both) compared to MRI findings.1T MRI is not useful for the diagnosis of GCA because of its low sensitivity. US and physical examination of temporal arteries had a better diagnostic accuracy. However, our data does not exclude a diagnostic role for higher-resolution MRI.
Published: 2008

49. Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials and Research group database

Author: Walker, U.A. Tyndall, A. Czirják, L. Denton, C. Farge-Bancel, D. Kowal-Bielecka, O. Müller-Ladner, U. Bocelli-Tyndall, C. Matucci-Cerinic, M. Riemekasten, G. Brückner, C. Airó, P. Scarsi, M. Scorza, R. Beretta, L. Cozzi, F. Tiso, F. Vonk, M.C. Van Den Hoogen, F.H.J. Wigley, F.M. Hummers, L. Nevskaya, T. Ananieva, L. Miniati, I. Tartaglia, N. Lomater, C. Balbir-Gurman, A. Braun-Moscovici, Y. Bambara, L.M. Caramaschi, P. Valentini, G. Ruocco, L. Krieg, T. Hunzelmann, N. Varjú, C. Carriera, P.E. Joven, B. Iannone, F. Lapadula, G. Kahan, A. Allanore, Y. Gabrielli, A. Imperatore, M. Scheja, A. Wollheim, F. Damjanov, N. Ostojic, P. Saar, P. Tarner, I.H. Kötter, I. Bombardieri, S. Bazzichi, L. Del Papa, N. Comina, D.P. Lo Monaco, A. La Corte, R. Hachulla, E. Launay, D. Distler, O. Ciurea, A. Sierakowski, S. Mitchell, H. Silver, R.M. Krasowska, D. Michalska-Jakubus, M. Tikly, M. Aboo, N. Worm, M. Klaus, P. Rovenský, J. Lukáčová, O. Rozman, B. Sipek, A. Clemente-Coelho, P. Shoenfeld, Y. Langewitch, P. Da Silva José, A.P. Salvador, M.J. Kuhn, A. Erdmann, G. Bečvář, R. Friedl, E. Graninger, W. Riccieri, V. Caporali, R. Montecucco, C. Vlachoyiannopoulos, P. Distler, M. Reich, K. Majdan, M. Wielosz, E. Rednic, S. Van Laar, J.M. Heitmann, S. Bruckner, A. Himsel, A. Riemann, J. Meyringer, R. Müller, A. Martinovic, D. Radic, M. Sticherling, M. Szekanecz, Z. Szücs, G. Giacomelli, R. Marrelli, A. Stamenkovic, B. Stankovic, A. Aringer, M. Smolen, J.S. Kucharz, E.J. Kotulska, A.T. Jablonska, S. Blasczik, M. Jun, J.-B. Mallia, C. Coleiro, B. Santamaria, V.O. Hinrichs, R. Nielsen, H. Cossutta, R. Ionescu, R. Opris, D. Steinbrink, K. Grundt, B. Bajocchi, G. Jiří, Š. Lefebvre, P.G.D.L.P. Zea Mendoza, A.C. Ribi, C. Chizzolini, C. Wisłowska, M. Novak, S. Indiveri, F. Jacobsen, S. Frandsen, P.B. Gorska, I.Z. Tore Gran, J. Midtvedt, Ø. Ramos, F.O. Rajcevska, L.D. Bozinovski, G. Schöffel, D. Sunderkötter, C. Böhm, M. Morović-Vergles, J. Čulo, M.-I. Cutolo, M. Sulli, A. Derk, C.T. Jimenez, S.A. Siakka, P. Søndergaard, K. Stengaard-Pedersen, K. Cabane, J. Tiev, K.P. Mihai, C. Sfrent-Cornateanu, R. Jendro, M. Tuvik, P. Antivalle, M. Randisi, G. Seidel, M. Clarenbach, R. Simsek, I. Dinc, A. Inanc, M. Capraru, M.S. Capraru, D. Bañegil, I. Richter, J. Alhasani, S. Földvari, I. Pinto, S. Brandão, F. Mas, A.J.
Subjects: integumentary system, skin and connective tissue diseases
Abstract: Background: Systemic sclerosis (SSc) is a multisystem autoimmune disease, which is classified into a diffuse cutaneous (dcSSc) and a limited cutaneous (IcSSc) subset according to the skin involvement. In order to better understand the vascular, immunological and fibrotic processes of SSc and to guide its treatment, the EULAR Scleroderma Trials And Research (EUSTAR) group was formed in June 2004. Aims and methods: EUSTAR collects prospectively the Minimal Essential Data Set (MEDS) on all sequential patients fulfilling the American College of Rheumatology diagnostic criteria in participating centres. We aimed to characterise demographic, clinical and laboratory characteristics of disease presentation in SSc and analysed EUSTAR baseline visits. Results: In April 2006, a total of 3656 patients (1349 with dcSSc and 2101 with IcSSc) were enrolled in 102 centres and 30 countries. 1330 individuals had autoantibodies against Scl70 and 1106 against anticentromere antibodies. 87% of patients were women. On multivariate analysis, scleroderma subsets (dcSSc vs IcSSc), antibody status and age at onset of Raynaud's phenomenon, but not gender, were found to be independently associated with the prevalence of organ manifestations. Autoantibody status in this analysis was more closely associated with clinical manifestations than were SSc subsets. Conclusion: dcSSc and IcSSc subsets are associated with particular organ manifestations, but in this analysis the clinical distinction seemed to be superseded by an antibody-based classification in predicting some scleroderma complications. The EUSTAR MEDS database facilitates the analysis of clinical patterns in SSc, and contributes to the standardised assessment and monitoring of SSc internationally.
Published: 2007

50. Visual manifestations in an Italian population-based cohort of patients with giant cell arteritis

Author: Salvarani, C., Luca Cimino, Macchioni, P., Catanoso, Mg, Restuccia, G., Opipotone, N., Bajocchi, G., Cantini, F., and Boiardi, L.
Published: 2004

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