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Introduction: The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods: Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results: Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (p<0.001). Patients with dcSSc were more likely to be younger and male (p<0.001) and have shorter disease duration, more calcinosis, tendon friction rubs and synovitis (all p<0.001). Interstitial lung disease (ILD) occurred more frequently in dcSSc but prevalence of pulmonary arterial hypertension (PAH) was similar in both subtypes. More deaths occurred among SCORE patients who had the shortest median survival (p<0.001). The survival of patients with early disease, males and those with dcSSc was shorter than that of patients with prevalent disease, female gender and lcSSc, respectively. SSc-related complications accounted for more than 50% of deaths, with PAH and ILD being the most common. Conclusions: This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.
What have multicentre registries across the world taught us about the disease features of systemic sclerosis?
Proudman S. M.;Huq M.;Stevens W.;Wilson M. E.;Sahhar J.;Baron M.;Hudson M.;Pope J.;Allanore Y.;Distler O.;Kowal-Bielecka O.;Matucci-Cerinic M.;H. L. Low A.;Teng G. G.;Law W. G.;Santosa A.;Nikpour M.;Hill C.;Lester S.;Nash P.;Ngian G. -S.;Proudman S.;Rischmueller M.;Roddy J.;Strickland G.;Thakkar V.;Walker J.;Zochling J.;Pope J.;Baron M.;Markland J.;Robinson D.;Jones N.;Khalidi N.;Docherty P.;Kaminska E.;Masetto A.;Sutton E.;Mathieu J. -P.;Hudson M.;Ligier S.;Grodzicky T.;LeClercq S.;Thorne C.;Gyger G.;Smith D.;Fortin P. R.;Larche M.;Abu-Hakima M.;Rodriguez-Reyna T. S.;Cabral A. R.;Fritzler M.;Avouac J.;Walker U. A.;Guiducci S.;Riemekasten G.;Air P.;Hachulla E.;Valentini G.;Carreira P. E.;Cozzi F.;Gurman A. B.;Braun-Moscovici Y.;Damjanov N.;Ananieva L. P.;Scorza R.;Jimenez S.;Busquets J.;Li M.;Muller-Ladner U.;Maurer B.;Tyndall A.;Lapadula G.;Iannone F.;Becvar R.;Sierakowsky S.;Cutolo M.;Sulli A.;Cuomo G.;Vettori S.;Rednic S.;Nicoara I.;Vlachoyiannopoulos P.;Montecucco C.;Caporali R.;Novak S.;Czirjak L.;Varju C.;Chizzolini C.;Kucharz E. J.;Kotulska A.;Kopec-Medrek M.;Widuchowska M.;Rozman B.;Mallia C.;Coleiro B.;Gabrielli A.;Farge D.;Hij A.;Hesselstrand R.;Scheja A.;Wollheim F.;Martinovic D.;Govoni M.;Lo Monaco A.;Hunzelmann N.;Pellerito R.;Bambara L. M.;Caramaschi P.;Black C.;Denton C.;Henes J.;Santamaria V. O.;Heitmann S.;Krasowska D.;Seidel M.;Oleszowsky M.;Burkhardt H.;Himsel A.;Salvador M. J.;Stamenkovic B.;Stankovic A.;Tikly M.;Starovoytova M. N.;Engelhart M.;Strauss G.;Nielsen H.;Damgaard K.;Szucs G.;Mendoza A. Z.;de la Puente Buijdos C.;Giraldo W. A. S.;Midtvedt O.;Garen T.;Launay D.;Valesini G.;Riccieri V.;Ionescu R. M.;Opris D.;Groseanu L.;Wigley F. M.;Mihai C. M.;Cornateanu R. S.;Ionitescu R.;Gherghe A. M.;Gorga M.;Dobrota R.;Bojinca M.;Schett G.;Distler J. H.;Meroni P.;Zeni S.;Mouthon L.;De Keyser F.;Smith V.;Cantatore F. P.;Corrado A.;Ullman S.;Iversen L.;Pozzi M. R.;Eyerich K.;Hein R.;Knott E.;Szechinski J.;Wiland P.;Szmyrka-Kaczmarek M.;Sokolik R.;Morgiel E.;Krummel-Lorenz B.;Saar P.;Aringer M.;Gunther C.;Anic B.;Baresic M.;Mayer M.;Radominski S. C.;de Souza Muller C.;Azevedo V. F.;Agachi S.;Groppa L.;Chiaburu L.;Russu E.;Zenone T.;Stebbings S.;Highton J.;Stamp L.;Chapman P.;O'Donnell J.;Solanki K.;Doube A.;Veale D.;O'Rourke M.;Loyo E.;Rosato E.;Pisarri S.;Tanaseanu C. -M.;Popescu M.;Dumitrascu A.;Tiglea I.;Chirieac R.;Ancuta C.;Furst D. E.;Kafaja S.;Garcia de la Pena Lefebvre P.;Rubio S. R.;Exposito M. V.;Sibilia J.;Chatelus E.;Gottenberg J. E.;Chifflot H.;Litinsky I.;Venalis A.;Butrimiene I.;Venalis P.;Rugiene R.;Karpec D.;Kerzberg E.;Montoya F.;Cosentino V.;Low A. H. L.;Teng G.;Chan G.;Lim A. Y. N.;Ng S. C.
2017-01-01
Abstract
Introduction: The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods: Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results: Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (p<0.001). Patients with dcSSc were more likely to be younger and male (p<0.001) and have shorter disease duration, more calcinosis, tendon friction rubs and synovitis (all p<0.001). Interstitial lung disease (ILD) occurred more frequently in dcSSc but prevalence of pulmonary arterial hypertension (PAH) was similar in both subtypes. More deaths occurred among SCORE patients who had the shortest median survival (p<0.001). The survival of patients with early disease, males and those with dcSSc was shorter than that of patients with prevalent disease, female gender and lcSSc, respectively. SSc-related complications accounted for more than 50% of deaths, with PAH and ILD being the most common. Conclusions: This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11571/1332406
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simulazione ASN
Il report seguente simula gli indicatori relativi alla propria produzione scientifica in relazione alle soglie ASN 2023-2025 del proprio SC/SSD. Si ricorda che il superamento dei valori soglia (almeno 2 su 3) è requisito necessario ma non sufficiente al conseguimento dell'abilitazione. La simulazione si basa sui dati IRIS e sugli indicatori bibliometrici alla data indicata e non tiene conto di eventuali periodi di congedo obbligatorio, che in sede di domanda ASN danno diritto a incrementi percentuali dei valori. La simulazione può differire dall'esito di un’eventuale domanda ASN sia per errori di catalogazione e/o dati mancanti in IRIS, sia per la variabilità dei dati bibliometrici nel tempo. Si consideri che Anvur calcola i valori degli indicatori all'ultima data utile per la presentazione delle domande.
La presente simulazione è stata realizzata sulla base delle specifiche raccolte sul tavolo ER del Focus Group IRIS coordinato dall’Università di Modena e Reggio Emilia e delle regole riportate nel DM 589/2018 e allegata Tabella A. Cineca, l’Università di Modena e Reggio Emilia e il Focus Group IRIS non si assumono alcuna responsabilità in merito all’uso che il diretto interessato o terzi faranno della simulazione. Si specifica inoltre che la simulazione contiene calcoli effettuati con dati e algoritmi di pubblico dominio e deve quindi essere considerata come un mero ausilio al calcolo svolgibile manualmente o con strumenti equivalenti.
