PT  - JOURNAL ARTICLE
AU  - Anthony J Tyndall
AU  - Bettina Bannert
AU  - Madelon Vonk
AU  - Paolo Airò
AU  - Franco Cozzi
AU  - Patricia E Carreira
AU  - Dominique Farge Bancel
AU  - Yannick Allanore
AU  - Ulf Müller-Ladner
AU  - Oliver Distler
AU  - Florenzo Iannone
AU  - Raffaele Pellerito
AU  - Margarita Pileckyte
AU  - Irene Miniati
AU  - Lidia Ananieva
AU  - Alexandra Balbir Gurman
AU  - Nemanja Damjanov
AU  - Adelheid Mueller
AU  - Gabriele Valentini
AU  - Gabriela Riemekasten
AU  - Mohammed Tikly
AU  - Laura Hummers
AU  - Maria JS Henriques
AU  - Paola Caramaschi
AU  - Agneta Scheja
AU  - Blaz Rozman
AU  - Evelien Ton
AU  - Gábor Kumánovics
AU  - Bernard Coleiro
AU  - Eva Feierl
AU  - Gabriella Szucs
AU  - Carlos Alberto Von Mühlen
AU  - Valeria Riccieri
AU  - Srdan Novak
AU  - Carlo Chizzolini
AU  - Anna Kotulska
AU  - Christopher Denton
AU  - Paulo C Coelho
AU  - Ina Kötter
AU  - Ismail Simsek
AU  - Paloma García de la Pena Lefebvre
AU  - Eric Hachulla
AU  - James R Seibold
AU  - Simona Rednic
AU  - Jiří Štork
AU  - Jadranka Morovic-Vergles
AU  - Ulrich A Walker
TI  - Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database
AID  - 10.1136/ard.2009.114264
DP  - 2010 Oct 01
TA  - Annals of the Rheumatic Diseases
PG  - 1809--1815
VI  - 69
IP  - 10
4099  - http://ard.bmj.com/content/69/10/1809.short
4100  - http://ard.bmj.com/content/69/10/1809.full
SO  - Ann Rheum Dis2010 Oct 01; 69
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.