RT Journal Article
SR Electronic
T1 Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 1809
OP 1815
DO 10.1136/ard.2009.114264
VO 69
IS 10
A1 Anthony J Tyndall
A1 Bettina Bannert
A1 Madelon Vonk
A1 Paolo Airò
A1 Franco Cozzi
A1 Patricia E Carreira
A1 Dominique Farge Bancel
A1 Yannick Allanore
A1 Ulf Müller-Ladner
A1 Oliver Distler
A1 Florenzo Iannone
A1 Raffaele Pellerito
A1 Margarita Pileckyte
A1 Irene Miniati
A1 Lidia Ananieva
A1 Alexandra Balbir Gurman
A1 Nemanja Damjanov
A1 Adelheid Mueller
A1 Gabriele Valentini
A1 Gabriela Riemekasten
A1 Mohammed Tikly
A1 Laura Hummers
A1 Maria JS Henriques
A1 Paola Caramaschi
A1 Agneta Scheja
A1 Blaz Rozman
A1 Evelien Ton
A1 Gábor Kumánovics
A1 Bernard Coleiro
A1 Eva Feierl
A1 Gabriella Szucs
A1 Carlos Alberto Von Mühlen
A1 Valeria Riccieri
A1 Srdan Novak
A1 Carlo Chizzolini
A1 Anna Kotulska
A1 Christopher Denton
A1 Paulo C Coelho
A1 Ina Kötter
A1 Ismail Simsek
A1 Paloma García de la Pena Lefebvre
A1 Eric Hachulla
A1 James R Seibold
A1 Simona Rednic
A1 Jiří Štork
A1 Jadranka Morovic-Vergles
A1 Ulrich A Walker
YR 2010
UL http://ard.bmj.com/content/69/10/1809.abstract
AB Objectives To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan–Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. Results Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). Conclusion Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.