477 results on '"Giuggioli D"'
Search Results
2. AB1183 THE USE OF MYCOPHENOLATE MOFETIL IN LIMITED CUTANEOUS SYSTEMIC SCLEROSIS IS ASSOCIATED WITH A REDUCED RISK OF VASCULAR COMPLICATIONS REQUIRING PHARMACOLOGICAL INTERVENTION UPGRADES
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De Lorenzis, E., primary, Bosello, S. L., additional, Cacciapaglia, F., additional, De Angelis, R., additional, Cipolletta, E., additional, Natalello, G., additional, D’agostino, M. A., additional, Codullo, V., additional, De Luca, G., additional, Giuggioli, D., additional, Ingegnoli, F., additional, Riccieri, V., additional, Ferri, C., additional, and Matucci-Cerinic, M., additional
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- 2024
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3. POS1479-HPR TRAVEL DISTANCE TO TERTIARY HEALTHCARE REFLECTS DISEASE SEVERITY AND MEDICATION ACCESS IN SYSTEMIC SCLEROSIS PATIENTS: INSIGHTS FROM THE ITALIAN SPRING REGISTRY
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De Lorenzis, E., primary, Natalello, G., additional, Cacciapaglia, F., additional, De Angelis, R., additional, Cipolletta, E., additional, D’agostino, M. A., additional, Codullo, V., additional, De Luca, G., additional, Giuggioli, D., additional, Ingegnoli, F., additional, Riccieri, V., additional, Ferri, C., additional, Matucci-Cerinic, M., additional, and Bosello, S. L., additional
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- 2024
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4. POS0604 JAKi’S SURVIVAL RATE AND PREDICTORS OF DISCONTINUATION IN A COHORT OF PATIENTS WITH RHEUMATOID ARTHRITIS
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Larosa, M., primary, Becciolini, A., additional, Parisi, S., additional, Donato, E. DI, additional, Camellino, D., additional, Adorni, G., additional, Lucchini, G., additional, Santilli, D., additional, Fusaro, E., additional, Ditto, M. C., additional, Lo Gullo, A., additional, Paroli, M., additional, Caccavale, R., additional, Volpe, A., additional, Marchetta, A., additional, Raffeiner, B., additional, Celletti, E., additional, Penta, M. DI, additional, Sabatini, E., additional, Cipollone, F., additional, Reta, M., additional, Addimanda, O., additional, Magnani, M., additional, Visalli, E., additional, Foti, R., additional, Amato, G., additional, De Lucia, F., additional, Farina, A., additional, Girelli, F., additional, Bernardi, S., additional, Colina, M., additional, Andracco, R., additional, Mansueto, N., additional, Ferrero, G., additional, Del Medico, P., additional, Molica Colella, A., additional, Franchina, V., additional, Molica Colella, F., additional, Lumetti, F., additional, Sandri, G., additional, Salvarani, C., additional, Giuggioli, D., additional, Priora, M., additional, Serale, F., additional, Ianniello, A., additional, Nucera, V., additional, Ometto, F., additional, Giampietro, C., additional, Bravi, E., additional, Platè, I., additional, Arrigoni, E., additional, Mascella, F., additional, Focherini, M. C., additional, Bezzi, A., additional, Scolieri, P., additional, Bruzzese, V., additional, Ravagnani, V., additional, Rovera, G., additional, Fiorenza, A., additional, Vitetta, R., additional, and Ariani, A., additional
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- 2024
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5. OP0265 RITUXIMAB RETENTION RATE IN SYSTEMIC SCLEROSIS: A REAL-LIFE ITALIAN MULTICENTER STUDY
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De Luca, G., primary, De Lorenzis, E., additional, Campochiaro, C., additional, Cacciapaglia, F., additional, Del Papa, N., additional, Zanatta, E., additional, Airo’, P., additional, Lazzaroni, M. G., additional, Giuggioli, D., additional, De Santis, M., additional, Alonzi, G., additional, Stano, S., additional, Binda, M., additional, Moccaldi, B., additional, Tonutti, A., additional, Cavalli, S., additional, Iannone, F., additional, D’ Agostino, M. A., additional, Dagna, L., additional, Matucci-Cerinic, M., additional, and Bosello, S. L., additional
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- 2024
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6. POS0826 PREDICTORS OF RESPONSE AND LONG-TERM EFFICACY AND SAFETY OF NINTEDANIB IN SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE: DATA FROM AN ITALIAN MULTICENTRE STUDY
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Campochiaro, C., primary, De Luca, G., additional, Lazzaroni, M. G., additional, Armentaro, G., additional, Ruaro, B., additional, Stanziola, A., additional, Benfaremo, D., additional, De Lorenzis, E., additional, Moccaldi, B., additional, Bonomi, F., additional, Bianchessi, L. M., additional, Iannone, F., additional, Cacciapaglia, F., additional, Spinella, A., additional, Guiducci, S., additional, Codullo, V., additional, Bosello, S. L., additional, Cuomo, G., additional, Zanatta, E., additional, Confalonieri, P., additional, Del Papa, N., additional, Airo’, P., additional, Moroncini, G., additional, Giuggioli, D., additional, Dagna, L., additional, and Matucci-Cerinic, M., additional
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- 2024
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7. AB0595 DIFFERENCES BETWEEN SELECTIVE AND UNSELECTIVE JAKi: RESULTS FROM A MULTICENTRIC, REAL LIFE STUDY CARRIED OUT ON PATIENTS WITH RA
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Larosa, M., primary, Camellino, D., additional, Becciolini, A., additional, Donato, E. DI, additional, Adorni, G., additional, Lucchini, G., additional, Santilli, D., additional, Arrigoni, E., additional, Bravi, E., additional, Platè, I., additional, Bezzi, A., additional, Focherini, M. C., additional, Mascella, F., additional, Bruzzese, V., additional, Scolieri, P., additional, Parisi, S., additional, Ditto, M. C., additional, Fusaro, E., additional, Ravagnani, V., additional, Rovera, G., additional, Fiorenza, A., additional, Vitetta, R., additional, Marchetta, A., additional, Volpe, A., additional, Raffeiner, B., additional, Celletti, E., additional, Penta, M. DI, additional, Sabatini, E., additional, Cipollone, F., additional, Ometto, F., additional, Giampietro, C., additional, Nucera, V., additional, Ianniello, A., additional, Serale, F., additional, Priora, M., additional, Giuggioli, D., additional, Salvarani, C., additional, Sandri, G., additional, Lumetti, F., additional, Molica Colella, F., additional, Franchina, V., additional, Molica Colella, A., additional, Del Medico, P., additional, Caccavale, R., additional, Paroli, M., additional, Ferrero, G., additional, Mansueto, N., additional, Andracco, R., additional, Colina, M., additional, Bernardi, S., additional, Girelli, F., additional, Farina, A., additional, Foti, R., additional, De Lucia, F., additional, Amato, G., additional, Lo Gullo, A., additional, Magnani, M., additional, Addimanda, O., additional, Reta, M., additional, and Ariani, A., additional
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- 2024
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8. AB1197 THE PREDICTIVE ROLE OF THE TAPSE/sPAP RATIO FOR CARDIOVASCULAR EVENTS AND MORTALITY IN SYSTEMIC SCLEROSIS COMPLICATED BY PULMONARY HYPERTENSION
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De Pinto, M., primary, Coppi, F., additional, Spinella, A., additional, Pagnoni, G., additional, Morgante, V., additional, Macripo’, P., additional, Guerra, A. F., additional, Secchi, O., additional, Orlandi, M., additional, Amati, G., additional, Lumetti, F., additional, Sandri, G., additional, Rossi, R., additional, Boriani, G., additional, Mattioli, A. V., additional, Ferri, C., additional, and Giuggioli, D., additional
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- 2024
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9. Cryofibrinogen: A possible risk factor in systemic sclerosis
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Natali, P., primary, Debbia, D., additional, Spinella, A., additional, Amati, G., additional, De Pinto, M., additional, Simonini, C., additional, Berretti, R., additional, Trenti, T., additional, Mascia, M.T., additional, Giuggioli, D., additional, and Sandri, G., additional
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- 2024
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10. Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC)
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Quartuccio, L, Bortoluzzi, A, Scire, C, Marangoni, A, Del Frate, G, Treppo, E, Castelnovo, L, Saccardo, F, Zani, R, Candela, M, Fraticelli, P, Mazzaro, C, Renoldi, P, Scaini, P, Filippini, D, Visentini, M, Scarpato, S, Giuggioli, D, Mascia, M, Sebastiani, M, Zignego, A, Lauletta, G, Fiorilli, M, Casato, M, Ferri, C, Pietrogrande, M, Pioltelli, P, De Vita, S, Monti, G, Galli, M, Quartuccio L., Bortoluzzi A., Scire C. A., Marangoni A., Del Frate G., Treppo E., Castelnovo L., Saccardo F., Zani R., Candela M., Fraticelli P., Mazzaro C., Renoldi P., Scaini P., Filippini D. A., Visentini M., Scarpato S., Giuggioli D., Mascia M. T., Sebastiani M., Zignego A. L., Lauletta G., Fiorilli M., Casato M., Ferri C., Pietrogrande M., Pioltelli P. E., De Vita S., Monti G., Galli M., Quartuccio, L, Bortoluzzi, A, Scire, C, Marangoni, A, Del Frate, G, Treppo, E, Castelnovo, L, Saccardo, F, Zani, R, Candela, M, Fraticelli, P, Mazzaro, C, Renoldi, P, Scaini, P, Filippini, D, Visentini, M, Scarpato, S, Giuggioli, D, Mascia, M, Sebastiani, M, Zignego, A, Lauletta, G, Fiorilli, M, Casato, M, Ferri, C, Pietrogrande, M, Pioltelli, P, De Vita, S, Monti, G, Galli, M, Quartuccio L., Bortoluzzi A., Scire C. A., Marangoni A., Del Frate G., Treppo E., Castelnovo L., Saccardo F., Zani R., Candela M., Fraticelli P., Mazzaro C., Renoldi P., Scaini P., Filippini D. A., Visentini M., Scarpato S., Giuggioli D., Mascia M. T., Sebastiani M., Zignego A. L., Lauletta G., Fiorilli M., Casato M., Ferri C., Pietrogrande M., Pioltelli P. E., De Vita S., Monti G., and Galli M.
- Abstract
Cryoglobulinemic vasculitis (CV) or mixed cryoglobulinemic syndrome (MCS) is a systemic small-vessel vasculitis characterized by the proliferation of B-cell clones producing pathogenic immune complexes, called cryoglobulins. It is often secondary to hepatitis C virus (HCV), autoimmune diseases, and hematological malignancies. CV usually has a mild benign clinical course, but severe organ damage and life-threatening manifestations can occur. Recently, evidence in favor of rituximab (RTX), an anti-CD 20 monoclonal antibody, is emerging in CV: nevertheless, questions upon the safety of this therapeutic approach, especially in HCV patients, are still being issued and universally accepted recommendations that can help physicians in MCS treatment are lacking. A Consensus Committee provided a prioritized list of research questions to perform a systematic literature review (SLR). A search was made in Medline, Embase, and Cochrane library, updated to August 2021. Of 1227 article abstracts evaluated, 27 studies were included in the SLR, of which one SLR, 4 RCTs, and 22 observational studies. Seventeen recommendations for the management of mixed cryoglobulinemia with rituximab from the Italian Study Group of Cryoglobulinemia (GISC) were developed to give a valuable tool to the physician approaching RTX treatment in CV.
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- 2023
11. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry 'SPRING' of the Italian Society for Rheumatology
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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.
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- 2023
12. 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
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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.
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- 2023
13. 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
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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.
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- 2022
14. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology
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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.
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- 2022
15. The safety of iloprost in systemic sclerosis in a real-life experience
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Bellando-Randone, S., Bruni, C., Lepri, G., Fiori, G., Bartoli, F., Conforti, ML, Moggi-Pignone, A., Guiducci, S., Giuggioli, D., Colaci, M., Spinella, A., Ferri, C., and Matucci-Cerinic, M.
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- 2018
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16. AB0879 SAFETY AND EFFECTIVENESS OF RITUXIMAB TREATMENT FOR SYSTEMIC SCLEROSIS: AN UPDATE OF OUR CLINICAL EXPERIENCE
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Spinella, A., primary, Sandri, G., additional, De Pinto, M., additional, Greco, R., additional, Magnani, L., additional, Amati, G., additional, Lumetti, F., additional, Cerri, S., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2023
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17. AB0891 PROOF OF CONCEPT OF THE USE OF CO-DESIGNED 3D PRINTED ASSISTIVE DEVICES WITHIN AN OCCUPATIONAL THERAPY INTERVENTION IN SYSTEMIC SCLEROSIS
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Spinella, A., primary, Gherardini, F., additional, Dalpadulo, E., additional, Giuriati, D., additional, Bettelli, V., additional, De Pinto, M., additional, Amati, G., additional, Secchi, O., additional, Mascia, M. T., additional, Sandri, G., additional, and Giuggioli, D., additional
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- 2023
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18. OP0238 IMMUNOSUPPRESSION WITH TARGETED DMARDS REDUCES MORBIDITY AND MORTALITY IN PRE-CAPILLARY PULMONARY HYPERTENSION ASSOCIATED WITH SYSTEMIC SCLEROSIS: A EUSTAR ANALYSIS
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Bruni, C., primary, Tofani, L., additional, Fretheim, H., additional, Weber, Y., additional, Hachulla, E., additional, Carreira, P., additional, Giuggioli, D., additional, Airò, P., additional, Siegert, E., additional, Müller-Ladner, U., additional, Matucci-Cerinic, M., additional, Riemekasten, G., additional, Simeon Aznar, C. P., additional, De Vries-Bouwstra, J., additional, Saketkoo, L. A., additional, Distler, J., additional, Balbir-Gurman, A., additional, Castellví, I., additional, Zanatta, E., additional, Smith, V., additional, Denton, C. P., additional, Maurer, B., additional, Giollo, A., additional, Iannone, F., additional, Dagna, L., additional, Truchetet, M. E., additional, Kuwana, M., additional, Allanore, Y., additional, Tanaka, Y., additional, Martin, M., additional, Rosato, E., additional, Gheorghiu, A. M., additional, Del Galdo, F., additional, Solanki, K., additional, Vacca, A., additional, Resende, C., additional, Vieira, S., additional, Czirják, L., additional, Baresic, M., additional, Cantatore, F. P., additional, Riccieri, V., additional, Andréasson, K., additional, Chung, L., additional, Souza Muller, C., additional, Opris-Belinski, D., additional, Rednic, S., additional, Sfikakis, P., additional, Levy, Y., additional, Hsu, V., additional, Heitmann, S., additional, Henes, J., additional, Moroncini, G., additional, Iudici, M., additional, De Langhe, E., additional, Herrick, A., additional, Montecucco, C., additional, Hoffmann-Vold, A. M., additional, and Distler, O., additional
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- 2023
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19. AB0889 FILGOTINIB FOR THE TREATMENT OF SYSTEMIC SCLEROSIS OVERLAP RHEUMATOID ARTHRITIS: DATA FROM OUR PRELIMINARY EXPERIENCE
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De Pinto, M., primary, Spinella, A., additional, Gavioli, L., additional, Amati, G., additional, Rai, A., additional, Goatelli, F., additional, Macripo’, P., additional, Lumetti, F., additional, Secchi, O., additional, Sandri, G., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2023
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20. AB0840 ACUTE VASCULAR EFFECTS OF INTRAVENOUS ILOPROST IN SCLERODERMA PATIENTS CAN BE DETECTED BY POWER DOPPLER EXAMINATION OF THE FINGER SUBCUTANEOUS TISSUE
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Testoni, S., primary, Magnani, L., additional, Spinella, A., additional, Salvarani, C., additional, Giuggioli, D., additional, and Macchioni, P., additional
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- 2023
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21. AB1310 UTILIZATION AND ATTITUDES TOWARDS NAILFOLD VIDEOCAPILLAROSCOPY FOR THE ASSESSMENT OF MICROVASCULAR STATUS IN PATIENTS WITH LONG-COVID: A MULTICENTER ONLINE SURVEY
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Gotelli, E., primary, Ingegnoli, F., additional, De Angelis, R., additional, Guiducci, S., additional, Giuggioli, D., additional, Pizzorni, C., additional, Riccieri, V., additional, Sebastiani, M., additional, Sulli, A., additional, Smith, V., additional, and Cutolo, M., additional
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- 2023
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22. 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
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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.
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- 2023
23. Real-life efficacy and safety of nintedanib in systemic sclerosis-interstitial lung disease: data from an Italian multicentre study
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Campochiaro, C., De Luca, G., Lazzaroni, M. -G., Armentaro, G., Spinella, A., Vigone, B., Ruaro, B., Stanziola, A., Benfaremo, D., De Lorenzis, E., Moccaldi, B., Bosello, Silvia Laura, Cuomo, G., Beretta, Carlo Luigi, Zanatta, E., Giuggioli, D., Del Papa, N., Airo, P., Confalonieri, Marco, Moroncini, G., Dagna, L., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), Confalonieri M. (ORCID:0000-0002-3708-379X), Campochiaro, C., De Luca, G., Lazzaroni, M. -G., Armentaro, G., Spinella, A., Vigone, B., Ruaro, B., Stanziola, A., Benfaremo, D., De Lorenzis, E., Moccaldi, B., Bosello, Silvia Laura, Cuomo, G., Beretta, Carlo Luigi, Zanatta, E., Giuggioli, D., Del Papa, N., Airo, P., Confalonieri, Marco, Moroncini, G., Dagna, L., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), Beretta L. (ORCID:0000-0001-9924-2066), and Confalonieri M. (ORCID:0000-0002-3708-379X)
- Abstract
Introduction Nintedanib (NTD) has been shown to be effective in systemic sclerosis (SSc)-interstitial lung disease (ILD). Here we describe the efficacy and safety of NTD in a real-life setting. Methods Patients with SSc-ILD treated with NTD were retrospectively evaluated at 12 months prior to NTD introduction; at baseline and at 12 months after NTD introduction. The following parameters were recorded: SSc clinical features, NTD tolerability, pulmonary function tests and modified Rodnan skin score (mRSS). Results 90 patients with SSc-ILD (65% female, mean age 57.6±13.4 years, mean disease duration 8.8±7.6 years) were identified. The majority were positive for anti-topoisomerase I (75%) and 77 (85%) patients were on immunosuppressants. A significant decline in %predicted forced vital capacity (%pFVC) in the 12 months prior to NTD introduction was observed in 60%. At 12 months after NTD introduction, follow-up data were available for 40 (44%) patients and they showed a stabilisation in %pFVC (64±14 to 62±19, p=0.416). The percentage of patients with significant lung progression at 12 months was significantly lower compared with the previous 12 months (60% vs 17.5%, p=0.007). No significant mRSS change was observed. Gastrointestinal (GI) side effects were recorded in 35 (39%) patients. After a mean time of 3.6±3.1 months, NTD was maintained after dose adjustment in 23 (25%) patients. In nine (10%) patients, NTD was stopped after a median time of 4.5 (1-6) months. During the follow-up, four patients died. Conclusions In a real-life clinical scenario, NTD, in combination with immunosuppressants, may stabilise lung function. GI side effects are frequent and NTD dose adjustment may be necessary to retain the drug in patients with SSc-ILD.
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- 2023
24. Lung vascular changes as biomarkers of severity in systemic sclerosis-associated interstitial lung disease
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Bruni, C., Occhipinti, M., Pienn, M., Camiciottoli, G., Bartolucci, M., Bosello, Silvia Laura, Payer, C., Balint, Z., Larici, Anna Rita, Tottoli, A., Tofani, L., De Lorenzis, E., Lepri, G., Bellando-Randone, S., Spinella, A., Giuggioli, D., Masini, F., Cuomo, G., Lavorini, F., Colagrande, S., Olschewski, H., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), Larici A. R. (ORCID:0000-0002-1882-6244), Bruni, C., Occhipinti, M., Pienn, M., Camiciottoli, G., Bartolucci, M., Bosello, Silvia Laura, Payer, C., Balint, Z., Larici, Anna Rita, Tottoli, A., Tofani, L., De Lorenzis, E., Lepri, G., Bellando-Randone, S., Spinella, A., Giuggioli, D., Masini, F., Cuomo, G., Lavorini, F., Colagrande, S., Olschewski, H., Matucci-Cerinic, M., Bosello S. L. (ORCID:0000-0002-4837-447X), and Larici A. R. (ORCID:0000-0002-1882-6244)
- Abstract
OBJECTIVES: It has recently become possible to assess lung vascular and parenchymal changes quantitatively in thoracic CT images using automated software tools. We investigated the vessel parameters of patients with SSc, quantified by CT imaging, and correlated them with interstitial lung disease (ILD) features. METHODS: SSc patients undergoing standard of care pulmonary function testing and CT evaluation were retrospectively evaluated. CT images were analysed for ILD patterns and total pulmonary vascular volume (PVV) extents with Imbio lung texture analysis. Vascular analysis (volumes, numbers and densities of vessels, separating arteries and veins) was performed with an in-house developed software. A threshold of 5% ILD extent was chosen to define the presence of ILD, and commonly used cut-offs of lung function were adopted. RESULTS: A total of 79 patients [52 women, 40 ILD, mean age 56.2 (s.d. 14.2) years, total ILD extent 9.5 (10.7)%, PVV/lung volume % 2.8%] were enrolled. Vascular parameters for total and separated PVV significantly correlated with functional parameters and ILD pattern extents. SSc-associated ILD (SSc-ILD) patients presented with an increased number and volume of arterial vessels, in particular those between 2 and 4 mm of diameter, and with a higher density of arteries and veins of <6 mm in diameter. Considering radiological and functional criteria concomitantly, as well as the descriptive trends from the longitudinal evaluations, the normalized PVVs, vessel numbers and densities increased progressively with the increase/worsening of ILD extent and functional impairment. CONCLUSION: In SSc patients CT vessel parameters increase in parallel with ILD extent and functional impairment, and may represent a biomarker of SSc-ILD severity.
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- 2023
25. Impact of COVID-19 and vaccination campaign on 1,755 systemic sclerosis patients during first three years of pandemic. Possible risks for individuals with impaired immunoreactivity to vaccine, ongoing immunomodulating treatments, and disease-related lung involvement during the next pandemic phase
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Ferri, C., Raimondo, V., Giuggioli, D., Gragnani, L., Lorini, S., Dagna, L., Bosello, Silvia Laura, Foti, R., Riccieri, V., Guiducci, S., Cuomo, G., Tavoni, A., De Angelis, R., Cacciapaglia, F., Zanatta, E., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Pellegrini, R., Varcasia, G., De Santis, M., Selmi, C., Abignano, G., Caminiti, M., L'Andolina, M., Olivo, D., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Ruscitti, P., Urraro, T., Visentini, M., Bellando-Randone, S., Visalli, E., Testa, D., Sciascia, G., Masini, F., Pellegrino, G., Saccon, F., Balestri, E., Elia, G., Ferrari, S. M., Tonutti, A., Dall'Ara, F., Pagano Mariano, G., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Dal Bosco, Y., Di Cola, I., Scorpiniti, D., Fusaro, E., Ferrari, T., Gigliotti, P., Campochiaro, C., Francioso, F., Iandoli, C., Caira, V., Zignego, A. L., D'Angelo, S., Franceschini, F., Matucci-Cerinic, M., Giacomelli, R., Doria, A., Santini, Stefano Angelo, Fallahi, P., Iannone, F., Antonelli, A., Bosello S. L. (ORCID:0000-0002-4837-447X), Santini S. A. (ORCID:0000-0003-1956-5899), Ferri, C., Raimondo, V., Giuggioli, D., Gragnani, L., Lorini, S., Dagna, L., Bosello, Silvia Laura, Foti, R., Riccieri, V., Guiducci, S., Cuomo, G., Tavoni, A., De Angelis, R., Cacciapaglia, F., Zanatta, E., Cozzi, F., Murdaca, G., Cavazzana, I., Romeo, N., Codullo, V., Pellegrini, R., Varcasia, G., De Santis, M., Selmi, C., Abignano, G., Caminiti, M., L'Andolina, M., Olivo, D., Lubrano, E., Spinella, A., Lumetti, F., De Luca, G., Ruscitti, P., Urraro, T., Visentini, M., Bellando-Randone, S., Visalli, E., Testa, D., Sciascia, G., Masini, F., Pellegrino, G., Saccon, F., Balestri, E., Elia, G., Ferrari, S. M., Tonutti, A., Dall'Ara, F., Pagano Mariano, G., Pettiti, G., Zanframundo, G., Brittelli, R., Aiello, V., Dal Bosco, Y., Di Cola, I., Scorpiniti, D., Fusaro, E., Ferrari, T., Gigliotti, P., Campochiaro, C., Francioso, F., Iandoli, C., Caira, V., Zignego, A. L., D'Angelo, S., Franceschini, F., Matucci-Cerinic, M., Giacomelli, R., Doria, A., Santini, Stefano Angelo, Fallahi, P., Iannone, F., Antonelli, A., Bosello S. L. (ORCID:0000-0002-4837-447X), and Santini S. A. (ORCID:0000-0003-1956-5899)
- Abstract
Introduction: The impact of COVID-19 pandemic represents a serious challenge for ‘frail’ patients' populations with inflammatory autoimmune systemic diseases such as systemic sclerosis (SSc). We investigated the prevalence and severity of COVID-19, as well the effects of COVID-19 vaccination campaign in a large series of SSc patients followed for the entire period (first 38 months) of pandemic. Patients and method: This prospective survey study included 1755 unselected SSc patients (186 M, 1,569F; mean age 58.7 ± 13.4SD years, mean disease duration 8.8 ± 7.3SD years) recruited in part by telephone survey at 37 referral centers from February 2020 to April 2023. The following parameters were carefully evaluated: i. demographic, clinical, serological, and therapeutical features; ii. prevalence and severity of COVID-19; and iii. safety, immunogenicity, and efficacy of COVID-19 vaccines. Results: The prevalence of COVID-19 recorded during the whole pandemic was significantly higher compared to Italian general population (47.3 % vs 43.3 %, p < 0.000), as well the COVID-19-related mortality (1.91 % vs 0.72 %, p < 0.001). As regards the putative prognostic factors of worse outcome, COVID-19 positive patients with SSc-related interstitial lung involvement showed significantly higher percentage of COVID-19-related hospitalization compared to those without (5.85 % vs 1.73 %; p < 0.0001), as well as of mortality rate (2.01 % vs 0.4 %; p = 0.002). Over half of patients (56.3 %) received the first two plus one booster dose of vaccine; while a fourth dose was administered to 35.6 %, and only few of them (1.99 %) had five or more doses of vaccine. Of note, an impaired seroconversion was recorded in 25.6 % of individuals after the first 2 doses of vaccine, and in 8.4 % of patients also after the booster dose. Furthermore, the absence of T-cell immunoreactivity was observed in 3/7 patients tested by QuantiFERON® SARSCoV-2 Starter Set (Qiagen). The efficacy of vaccines, evalu
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- 2023
26. Nailfold capillaroscopy in the rheumatological current clinical practice in Italy: results of a national survey
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Ingegnoli, F., primary, Cornalba, M., additional, De Angelis, R., additional, Guiducci, S., additional, Giuggioli, D., additional, Pizzorni, C., additional, Riccieri, V., additional, Sebastiani, M., additional, Sulli, A., additional, and Cutolo, M., additional
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- 2022
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27. Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of scleroderma spectrum
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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.
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- 2020
28. NON-SURGICAL LOCAL TREATMENTS FOR DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW
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Campochiaro, C., Suliman, Y. A., Hughes, M., Schoones, J., Giuggioli, D., Moinzadeh, P., Maltez, N., Ross, L., Baron, M., Chung, L., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., Alunno, A., Campochiaro, C., Suliman, Y. A., Hughes, M., Schoones, J., Giuggioli, D., Moinzadeh, P., Maltez, N., Ross, L., Baron, M., Chung, L., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., and Alunno, A.
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- 2022
29. SYSTEMIC PHARMACOLOGICAL TREATMENT OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW
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Maltez, N., Ross, L., Hughes, M., Schoones, J., Baron, M., Chung, L., Campochiaro, C., Suliman, Y. A., Giuggioli, D., Moinzadeh, P., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., Alunno, A., Maltez, N., Ross, L., Hughes, M., Schoones, J., Baron, M., Chung, L., Campochiaro, C., Suliman, Y. A., Giuggioli, D., Moinzadeh, P., Allanore, Y., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., and Alunno, A.
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- 2022
30. SURGICAL MANAGEMENT OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW.
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Suliman, Y. A., Campochiaro, C., Hughes, M., Schoones, J., Giuggioli, D., Maltez, N., Moinzadeh, P., Ross, L., Chung, L., Allanore, Y., Baron, M., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., Alunno, A., Suliman, Y. A., Campochiaro, C., Hughes, M., Schoones, J., Giuggioli, D., Maltez, N., Moinzadeh, P., Ross, L., Chung, L., Allanore, Y., Baron, M., Denton, C. P., Distler, O., Frech, T., Furst, D., Khanna, D., Krieg, T., Kuwana, M., Matucci-Cerinic, M., Pope, J., and Alunno, A.
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- 2022
31. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology
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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.
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- 2022
32. Absent or suboptimal response to booster dose of COVID-19 vaccine in patients with autoimmune systemic diseases
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Ferri, C., Gragnani, L., Raimondo, V., Visentini, M., Giuggioli, D., Lorini, S., Foti, R., Cacciapaglia, F., Caminiti, M., Olivo, D., Cuomo, G., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Cavazzana, I., Ruscitti, P., Vadacca, M., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Bosco, Y. D., Amato, G., Masini, F., Mariano, G. P., Brittelli, R., Aiello, V., Scorpiniti, D., Rechichi, G., Varcasia, G., Monti, M., Elia, G., Franceschini, F., Casato, M., Ursini, F., Giacomelli, R., Fallahi, P., Santini, Stefano Angelo, Iannone, F., Salvarani, C., Zignego, A. L., Antonelli, A., Santini S. A. (ORCID:0000-0003-1956-5899), Ferri, C., Gragnani, L., Raimondo, V., Visentini, M., Giuggioli, D., Lorini, S., Foti, R., Cacciapaglia, F., Caminiti, M., Olivo, D., Cuomo, G., Pellegrini, R., Pigatto, E., Urraro, T., Naclerio, C., Tavoni, A., Puccetti, L., Cavazzana, I., Ruscitti, P., Vadacca, M., La Gualana, F., Cozzi, F., Spinella, A., Visalli, E., Bosco, Y. D., Amato, G., Masini, F., Mariano, G. P., Brittelli, R., Aiello, V., Scorpiniti, D., Rechichi, G., Varcasia, G., Monti, M., Elia, G., Franceschini, F., Casato, M., Ursini, F., Giacomelli, R., Fallahi, P., Santini, Stefano Angelo, Iannone, F., Salvarani, C., Zignego, A. L., Antonelli, A., and Santini S. A. (ORCID:0000-0003-1956-5899)
- Abstract
Autoimmune systemic diseases (ASD) show impaired immunogenicity to COVID-19 vaccines. Our prospective observational multicenter study aimed at evaluating the seroconversion elicited by COVID-19 vaccine over the entire vaccination cycle including the booster dose. Among 478 unselected ASD patients originally evaluated at the end of the first vaccination cycle (time 1), 344 individuals were re-evaluated after a 6-month period (time 2), and 244 after the booster vaccine dose (time 3). The immunogenicity of mRNA COVID-19 vaccines (BNT162b2 and mRNA-1273) was assessed by measuring serum IgG-neutralizing antibody (NAb) on samples obtained at the three time points in both patients and 502 age-matched controls. In the 244 ASD group that received booster vaccine and monitored over the entire follow-up, the mean serum NAb levels (time 1, 2, and 3: 696.8 ± 52.68, 370.8 ± 41.92, and 1527 ± 74.16SD BAU/mL, respectively; p < 0.0001) were constantly lower compared to controls (p < 0.0001), but they significantly increased after the booster dose compared to the first two measurements (p < 0.0001). The percentage of patients with absent/suboptimal response to vaccine significantly decreased after the booster dose compared to the first and second evaluations (time 1, 2, and 3: from 28.2% to 46.3%, and to 7.8%, respectively; p < 0.0001). Of note, the percentage of patients with absent/suboptimal response after the booster dose was significantly higher compared to controls (19/244, 7.8% vs 1/502, 0.2%; p < 0.0001). Similarly, treatment with immune-modifiers increased the percentage of patients exhibiting absent/suboptimal response (16/122, 13.1% vs 3/122, 2.46%; p = 0.0031). Overall, the above findings indicate the usefulness of booster vaccine administration in ASD patients. Moreover, the persistence of a significantly higher percentage of individuals without effective seroconversion (7.8%), even after the booster dose, warrants for careful monitoring of NAb levels in a
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- 2022
33. 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
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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.
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- 2022
34. Nailfold capillaroscopic changes in dermatomyositis and polymyositis
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Manfredi, A., Sebastiani, M., Cassone, G., Pipitone, N., Giuggioli, D., Colaci, M., Salvarani, C., and Ferri, C.
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- 2015
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35. POS1382 AN OVERVIEW OF NAILFOLD CAPILLAROSCOPY IN ITALY: RESULTS FROM A NATIONAL SURVEY
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Ingegnoli, F., primary, Cornalba, M., additional, De Angelis, R., additional, Guiducci, S., additional, Giuggioli, D., additional, Pizzorni, C., additional, Riccieri, V., additional, Sebastiani, M., additional, Sulli, A., additional, and Cutolo, M., additional
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- 2022
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36. POS0896 CRYOFIBRINOGEN-ASSOCIATED SYSTEMIC SCLEROSIS: POSSIBLE CORRELATIONS WITH SCLERODERMA DIGITAL ULCERS AND PULMONARY ARTERIAL HYPERTENSION
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Amati, G., primary, Spinella, A., additional, Sandri, G., additional, Natali, P., additional, Debbia, D., additional, Mascia, M. T., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2022
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37. POS0890 NINTEDANIB REAL-LIFE EFFICACY AND SAFETY IN SYSTEMIC SCLEROSIS (SSc)-INTERTISTIAL LUNG DISEASE (ILD): AN ITALIAN MULTICENTRE PRELIMINARY STUDY.
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Campochiaro, C., primary, De Luca, G., additional, Lazzaroni, M. G., additional, Armentaro, G., additional, Spinella, A., additional, Vigone, B., additional, Ruaro, B., additional, Stanziola, A., additional, Benfaremo, D., additional, De Lorenzis, E., additional, Benvenuti, F., additional, Bosello, S. L., additional, Moroncini, G., additional, Cuomo, G., additional, Confalonieri, M., additional, Beretta, L., additional, Zanatta, E., additional, Giuggioli, D., additional, Del Papa, N., additional, Airò, P., additional, Dagna, L., additional, and Matucci-Cerinic, M., additional
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- 2022
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38. POS0900 SYSTEMIC PHARMACOLOGICAL TREATMENT OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW
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Maltez, N., primary, Ross, L., additional, Hughes, M., additional, Schoones, J., additional, Baron, M., additional, Chung, L., additional, Campochiaro, C., additional, Suliman, Y. A., additional, Giuggioli, D., additional, Moinzadeh, P., additional, Allanore, Y., additional, Denton, C. P., additional, Distler, O., additional, Frech, T., additional, Furst, D., additional, Khanna, D., additional, Krieg, T., additional, Kuwana, M., additional, Matucci-Cerinic, M., additional, Pope, J., additional, and Alunno, A., additional
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- 2022
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39. POS0919 MOLECULAR AND BIOLOGICAL PATHWAYS OF BREAST CANCER IN PATIENTS WITH SYSTEMIC SCLEROSIS: IMMUNOHISTOCHEMICAL INVESTIGATIONS FROM THE SCLERO-BREAST STUDY
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Spinella, A., primary, Toss, A., additional, Isca, C., additional, De Pinto, M., additional, Vacchi, C., additional, Magnani, L., additional, Lumetti, F., additional, Macripo’, P., additional, Ficarra, G., additional, Fabbiani, L., additional, Iannone, A., additional, Gasparini, E., additional, Piana, S., additional, Cortesi, L., additional, De Santis, G., additional, Maiorana, A., additional, Dominici, M., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2022
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40. AB0722 One-year clinical experience on the use of Nintedanib in Systemic Sclerosis: safety profile.
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Magnani, L., primary, Spinella, A., additional, Testoni, S., additional, Bajocchi, G., additional, Dardani, L., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2022
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41. POS0918 TARGET-CONTROLLED INFUSION (TCI) WITH PROPOFOL AND REMIFENTANIL FOR PROCEDURAL SEDATION AND ANALGESIA (PSA) IN PATIENTS WITH SYSTEMIC SCLEROSIS (SSC) UNDERGOING AUTOLOGOUS FAT GRAFTING PROCEDURES
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Feminò, R., primary, Spinella, A., additional, Carratta, A., additional, De Pinto, M., additional, Starnoni, M., additional, Bertellini, E., additional, De Santis, G., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2022
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42. POS0921 DIGITAL GANGRENE IN SYSTEMIC SCLEROSIS: AUTOLOGOUS FAT GRAFTING AND BONE SHAVING TO AVOID AMPUTATION
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Starnoni, M., primary, Spinella, A., additional, Feminò, R., additional, De Pinto, M., additional, Dominici, M., additional, De Santis, G., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2022
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43. AB0132 STIMULATION OF SOLUBLE GUANYLATE CYCLASE (sGC) FOSTERS ANGIOGENESIS AND BLUNTS ENDOTHELIAL-TO-MESENCHYMAL TRANSITION (EndoMT) OF SYSTEMIC SCLEROSIS (SSc) DERMAL MICROVASCULAR ENDOTHELIAL CELLS
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Romano, E., primary, Rosa, I., additional, Fioretto, B. S., additional, Giuggioli, D., additional, Manetti, M., additional, and Matucci-Cerinic, M., additional
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- 2022
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44. AB0725 Scleroderma study group Emilia Romagna (Sclero-RER): real life use of prostacyclin analog. Preliminary data from a multicentric survey.
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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
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- 2022
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45. POS0898 SURGICAL MANAGEMENT OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW.
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Suliman, Y. A., primary, Campochiaro, C., additional, Hughes, M., additional, Schoones, J., additional, Giuggioli, D., additional, Maltez, N., additional, Moinzadeh, P., additional, Ross, L., additional, Chung, L., additional, Allanore, Y., additional, Baron, M., additional, Denton, C. P., additional, Distler, O., additional, Frech, T., additional, Furst, D., additional, Khanna, D., additional, Krieg, T., additional, Kuwana, M., additional, Matucci-Cerinic, M., additional, Pope, J., additional, and Alunno, A., additional
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- 2022
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46. POS0382 RNA-SEQUENCING ANALYSIS TO IDENTIFY POTENTIAL FUNCTIONAL PATHWAYS INVOLVED IN SYSTEMIC SCLEROSIS PATHOGENESIS: OUR PRELIMINARY EXPERIENCE
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Spinella, A., primary, Lo Tartaro, D., additional, Gibellini, L., additional, Starnoni, M., additional, de Pinto, M., additional, Amati, G., additional, De Santis, G., additional, Cossarizza, A., additional, Salvarani, C., additional, and Giuggioli, D., additional
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- 2022
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47. POS0888 NON-SURGICAL LOCAL TREATMENTS FOR DIGITAL ULCERS IN SYSTEMIC SCLEROSIS: A SYSTEMATIC LITERATURE REVIEW
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Campochiaro, C., primary, Suliman, Y. A., additional, Hughes, M., additional, Schoones, J., additional, Giuggioli, D., additional, Moinzadeh, P., additional, Maltez, N., additional, Ross, L., additional, Baron, M., additional, Chung, L., additional, Allanore, Y., additional, Denton, C. P., additional, Distler, O., additional, Frech, T., additional, Furst, D., additional, Khanna, D., additional, Krieg, T., additional, Kuwana, M., additional, Matucci-Cerinic, M., additional, Pope, J., additional, and Alunno, A., additional
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- 2022
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48. POS1267 LONG-TERM SURVEY STUDY OF THE IMPACT OF COVID-19 ON SYSTEMIC AUTOIMMUNE DISEASES. LOW DEATH RATE DESPITE THE INCREASED PREVALENCE OF SYMPTOMATIC INFECTION. ROLE OF PRE-EXISTING INTERSTITIAL LUNG DISEASE AND ONGOING TREATMENTS.
- Author
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Ferri, C., primary, Raimondo, V., additional, Gragnani, L., additional, Giuggioli, D., additional, Dagna, L., additional, Tavoni, A., additional, Ursini, F., additional, L’andolina, M., additional, Caso, F., additional, Ruscitti, P., additional, Caminiti, M., additional, Foti, R., additional, Riccieri, V., additional, Guiducci, S., additional, Pellegrini, R., additional, Zanatta, E., additional, Varcasia, G., additional, Olivo, D., additional, Gigliotti, P., additional, Cuomo, G., additional, Murdaca, G., additional, Cecchetti, R., additional, De Angelis, R., additional, Romeo, N., additional, Ingegnoli, F., additional, Cozzi, F., additional, Codullo, V., additional, Cavazzana, I., additional, Colaci, M., additional, Abignano, G., additional, De Santis, M., additional, Lubrano, E., additional, Fusaro, E., additional, Spinella, A., additional, Lumetti, F., additional, De Luca, G., additional, Bellando Randone, S., additional, Visalli, E., additional, Dal Bosco, Y., additional, Amato, G., additional, Giannini, D., additional, Bilia, S., additional, Masini, F., additional, Pellegrino, G., additional, Pigatto, E., additional, Generali, E., additional, Pagano Mariano, G., additional, Pettiti, G., additional, Zanframundo, G., additional, Brittelli, R., additional, Aiello, V., additional, Caminiti, R., additional, Scorpiniti, D., additional, Ferrari, T., additional, Campochiaro, C., additional, Brusi, V., additional, Fredi, M., additional, Moschetti, L., additional, Cacciapaglia, F., additional, Ferrari, S. M., additional, DI Cola, I., additional, Vadacca, M., additional, Lorusso, S., additional, Monti, M., additional, Lorini, S., additional, Paparo, S. R., additional, Ragusa, F., additional, Elia, G., additional, Mazzi, V., additional, Aprile, M. L., additional, Tasso, M., additional, Miccoli, M., additional, Bosello, S. L., additional, D’angelo, S., additional, Doria, A., additional, Franceschini, F., additional, Meliconi, R., additional, Matucci-Cerinic, M., additional, Iannone, F., additional, Giacomelli, R., additional, Salvarani, C., additional, Zignego, A. L., additional, Fallahi, P., additional, and Antonelli, A., additional
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- 2022
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49. AN OVERVIEW OF NAILFOLD CAPILLAROSCOPY IN ITALY: RESULTS FROM A NATIONAL SURVEY
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Ingegnoli, F, Cornalba, M, De Angelis, R, Guiducci, S, Giuggioli, D, Pizzorni, C, Riccieri, V, Sebastiani, M, Sulli, A, and Cutolo, M
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- 2022
50. COVID-19 vaccine hesitancy in systemic sclerosis
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Ciaffi, J., Giuggioli, D., Mari, A., D Onghia, M., Spinella, A., Brusi, V., Mancarella, L., Lisi, L., Jacopo Faranda Cordella, Freeman, D., Landini, M. P., Ferri, C., Meliconi, R., Ursini, F., Ciaffi J., Giuggioli D., Mari A., D Onghia M., Spinella A., Brusi V., Mancarella L., Lisi L., Faranda Cordella J., Freeman D., Landini M.P., Ferri C., Meliconi R., and Ursini F.
- Subjects
COVID-19 Vaccines ,Scleroderma, Systemic ,Rheumatology ,SARS-CoV-2 ,vaccine ,Immunology ,Vaccination ,Immunology and Allergy ,COVID-19 ,Humans ,systemic sclerosi - Abstract
None
- Published
- 2021
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