Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.

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Title:	Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.
Author(s):	Tyndall, A.J.; Bannert, B.; Vonk, M.C. ; Airo, P.; Cozzi, F.; Carreira, P.E.; Bancel, D.F.; Allanore, Y.; Muller-Ladner, U.; Distler, O.; Iannone, F.; Pellerito, R.; Pileckyte, M.; Miniati, I.; Ananieva, L.; Gurman, A.B.; Damjanov, N.; Mueller, A.; Valentini, G.; Riemekasten, G.; Tikly, M.; Hummers, L.; Henriques, M.J.; Caramaschi, P.; Scheja, A.; Rozman, B.; Ton, E.; Kumanovics, G.; Coleiro, B.; Feierl, E.; Szucs, G.; Muhlen, C.A. Von; Riccieri, V.; Novak, S.; Chizzolini, C.; Kotulska, A.; Denton, C.; Coelho, P.C.; Kotter, I.; Simsek, I.; Pena Lefebvre, P.G. de la; Hachulla, E.; Seibold, J.R.; Rednic, S.; Stork, J.; Morovic-Vergles, J.; Walker, U.A.
Publication year:	2010
Source:	Annals of the Rheumatic Diseases, vol. 69, iss. 10, (2010), pp. 1809-1815
ISSN:	0003-4967
DOI:	https://doi.org/10.1136/ard.2009.114264
Annotation:	1 oktober 2010
Publication type:	Article / Letter to editor
Please use this identifier to cite or link to this item : https://hdl.handle.net/2066/88008
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Subject:	N4i 4: Auto-immunity, transplantation and immunotherapy NCEBP 2: Evaluation of complex medical interventions NCEBP 5: Health care ethics
Organization:	Rheumatology
Journal title:	Annals of the Rheumatic Diseases
Volume:	vol. 69
Issue:	iss. 10
Page start:	p. 1809
Page end:	p. 1815
Abstract:	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.