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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
immunology and microbiology
What have multicentre registries across the world taught us about the disease features of systemic sclerosis?
Journal of Scleroderma and Related Disorders, Volume 2, No. 3, Year 2017
Notification
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Description
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. © 2017 Wichtig International
Authors & Co-Authors
Proudman, Susanna M.
Australia, Adelaide
Royal Adelaide Hospital
Australia, Adelaide
The University of Adelaide
Stevens, Wendy M.
Australia, Fitzroy
St. Vincent's Hospital Melbourne
Sahhar, Joanne M.
Australia, Clayton
Monash Health
Australia, Clayton
Monash University
Baron, Murray
Canada, Montreal
Sir Mortimer B. Davis Jewish General Hospital
Hudson, Marie Anne R.
Canada, Montreal
Sir Mortimer B. Davis Jewish General Hospital
Pope, Janet Elizabeth
Canada, London
Western University
Allanore, Yannick
France, Paris
Université Paris Cité
Distler, Oliver
Switzerland, Zurich
Universitatsspital Zurich
Kowal-Bielecka, Otylia M.
Poland, Bialystok
Uniwersytet Medyczny w Bialymstoku
Matucci-Cerinić, Marco M.
Italy, Florence
Università Degli Studi Di Firenze
Nikpour, Mandana
Australia, Fitzroy
St. Vincent's Hospital Melbourne
Hill, Catherine Louise
Unknown Affiliation
Lester, Susan Elizabeth
Unknown Affiliation
Nash, Peter T.
Unknown Affiliation
Rischmueller, Maureen
Unknown Affiliation
Roddy, Janet
Unknown Affiliation
Strickland, Gemma
Unknown Affiliation
Walker, Jennifer G.
Unknown Affiliation
Zochling, Jane M.
Unknown Affiliation
Khalidi, Nader A.
Unknown Affiliation
Thorne, Carter J.
Unknown Affiliation
Fortin, Paul R.
Unknown Affiliation
Larché, Maggie J.
Unknown Affiliation
Rodríguez-Reyna, Tatiana S.
Unknown Affiliation
Avouac, Jérôme
Unknown Affiliation
Walker, Ulrich Andreas
Unknown Affiliation
Guiducci, Serena
Unknown Affiliation
Riemekasten, Gabriela
Unknown Affiliation
Hachulla, Éric
Unknown Affiliation
Valentini, Gabriele
Unknown Affiliation
Carreira-Delgado, Patricia E.
Unknown Affiliation
Cozzi, Franco
Unknown Affiliation
Gurman, Alexandra Balbir
Unknown Affiliation
Braun-Moscovici, Yolanda
Unknown Affiliation
Damjanov, Nemanja S.
Unknown Affiliation
Ananyeva, L. P.
Unknown Affiliation
Scorza, Raffaella
Unknown Affiliation
Jimenez, Sergio A.
Unknown Affiliation
Li, Mengtao
Unknown Affiliation
Müller-Ladner, Ulf
Unknown Affiliation
Maurer, Britta
Unknown Affiliation
Tyndall, Alan D.
Unknown Affiliation
Lapadula, Giovanni
Unknown Affiliation
Iannone, Florenzo
Unknown Affiliation
Bečvář, Radim
Unknown Affiliation
Sierakowsky, Stanislaw
Unknown Affiliation
Cutolo, Maurizio
Unknown Affiliation
Sulli, Alberto
Unknown Affiliation
Cuomo, Giovanna
Unknown Affiliation
Vettori, Serena
Unknown Affiliation
Rednic, S.
Unknown Affiliation
Nicoara, Ileana
Unknown Affiliation
Vlachoyiannopoulos, Panayiotis G.
Unknown Affiliation
Caporali, R. Felice
Unknown Affiliation
Novak, Srđan
Unknown Affiliation
Czirjak, László
Unknown Affiliation
Varjú, Cecília
Unknown Affiliation
Chizzolini, Carlo
Unknown Affiliation
Kucharz, Eugeniusz Józef
Unknown Affiliation
Kotulska, Anna T.
Unknown Affiliation
Kopeć-Mędrek, Magdalena
Unknown Affiliation
Widuchowska, Małgorzata
Unknown Affiliation
Rozman, Blaž
Unknown Affiliation
Mallia, Carmel M.
Unknown Affiliation
Coleiro, Bernard
Unknown Affiliation
Gabrielli, Armando
Unknown Affiliation
Farge, Dominique
Unknown Affiliation
Hij, Adrian
Unknown Affiliation
Hesselstrand, Roger
Unknown Affiliation
Scheja, Agneta
Unknown Affiliation
Wollheim, Frank A.
Unknown Affiliation
Martinović, Duška
Unknown Affiliation
Govoni, Marcello
Unknown Affiliation
Lo Monaco, Andrea
Unknown Affiliation
Hunzelmann, Nicolas
Unknown Affiliation
Pellerito, Raffaele A.
Unknown Affiliation
Bambara, Lisa Maria
Unknown Affiliation
Caramaschi, Paola
Unknown Affiliation
Black, Carol Mary
Unknown Affiliation
Denton, Christopher Paul
Unknown Affiliation
Henes, Jörg Christoph
Unknown Affiliation
Santamaría, Vera Ortiz
Unknown Affiliation
Heitmann, Stefan
Unknown Affiliation
Krasowska, Dorota Maria
Unknown Affiliation
Seidel, Matthias F.
Unknown Affiliation
Oleszowsky, Mara
Unknown Affiliation
Burkhardt, Harald
Unknown Affiliation
Himsel, Andrea
Unknown Affiliation
Salvador, Maria João
Unknown Affiliation
Stamenković, Bojana N. N.
Unknown Affiliation
Stankovic, Aleksandra M.
Unknown Affiliation
Tikly, Mohammed
Unknown Affiliation
Starovoytova, M. N.
Unknown Affiliation
Engelhart, Merete
Unknown Affiliation
Strauss, Gitte Irene
Unknown Affiliation
Nielsen, Henrik P.
Unknown Affiliation
Damgaard, Kirsten
Unknown Affiliation
Szűcs, Gabriella
Unknown Affiliation
Mendoza, Antonio Zea
Unknown Affiliation
de la Puente Buijdos, Carlos
Unknown Affiliation
Sifuentes-Giraldo, Walter Alberto
Unknown Affiliation
Midtvedt, Øyvind
Unknown Affiliation
Garen, Torhild
Unknown Affiliation
Launay, David
Unknown Affiliation
Riccieri, Valeria
Unknown Affiliation
Ionescu, Ruxandra Maria
Unknown Affiliation
Opriş-Belinski, Daniela
Unknown Affiliation
Groșeanu, Laura
Unknown Affiliation
Wigley, Fredrick M.
Unknown Affiliation
Mihai, Carina Marina
Unknown Affiliation
Cornǎţeanu, Roxana Sfrenţ
Unknown Affiliation
Ionitescu, Rǎzvan C.
Unknown Affiliation
Gherghe, Ana Maria
Unknown Affiliation
Dobrotă, Rucsandra
Unknown Affiliation
Schett, Georg Andreas
Unknown Affiliation
Distler, Jor̈g H.W.
Unknown Affiliation
Meroni, Pier Luigi
Unknown Affiliation
Mouthon, Luc
Unknown Affiliation
de Keyser, Filip M.
Unknown Affiliation
Smith, Vanessa
Unknown Affiliation
Cantatore, Francesco Paolo
Unknown Affiliation
Ullman, Susanne
Unknown Affiliation
Iversen, Line Vinderslev
Unknown Affiliation
Pozzi, Maria Rosa
Unknown Affiliation
Eyerich, Kilian
Unknown Affiliation
Hein, Ruediger
Unknown Affiliation
Knott, Elisabeth
Unknown Affiliation
Szechiński, Jacek
Unknown Affiliation
Wiland, Piotr
Unknown Affiliation
Szmyrka, Magdalena
Unknown Affiliation
Sokolik, Renata
Unknown Affiliation
Morgiel, Ewa
Unknown Affiliation
Krummel-Lorenz, Brigitte
Unknown Affiliation
Saar, Petra
Unknown Affiliation
Aringer, Martin
Unknown Affiliation
Günther, Claudia H.
Unknown Affiliation
Anić, Branimir B.
Unknown Affiliation
Barešić, Marko
Unknown Affiliation
Mayer, Miroslav
Unknown Affiliation
Radominski, Sebastião Cézar
Unknown Affiliation
de Souza Müller, Carolina
Unknown Affiliation
Azevedo, V. F.
Unknown Affiliation
Zénone, Thierry
Unknown Affiliation
Stebbings, Simon M.
Unknown Affiliation
Stamp, Lisa K.
Unknown Affiliation
Solanki, Kamal Kishor
Unknown Affiliation
Veale, Douglas James
Unknown Affiliation
Loyo, Esthela
Unknown Affiliation
Rosato, Edoardo
Unknown Affiliation
Tǎnǎseanu, Cristina Mihaela
Unknown Affiliation
Ancuța, Codrina Michaela
Unknown Affiliation
Kafaja, Suzanne
Unknown Affiliation
Garcia-De-La-Peña-Lefebvre, P.
Unknown Affiliation
Sibilia, Jean
Unknown Affiliation
Chatelus, Emmanuel
Unknown Affiliation
Gottenberg, Jacques Éric
Unknown Affiliation
Litinsky, Ira
Unknown Affiliation
Venalis, Algirdas
Unknown Affiliation
Butrimienė, Irena
Unknown Affiliation
Karpec, Diana
Unknown Affiliation
Kerzberg, Eduardo Mario
Unknown Affiliation
Cosentino, Vanesa Laura
Unknown Affiliation
Statistics
Citations: 19
Authors: 162
Affiliations: 14
Identifiers
Doi:
10.5301/jsrd.5000256
ISSN:
23971983
Research Areas
Noncommunicable Diseases
Study Design
Cross Sectional Study
Cohort Study
Study Approach
Quantitative
Participants Gender
Male
Female