Advanced

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

Tyndall, Anthony J.; Bannert, Bettina; Vonk, Madelon; Airo, Paolo; Cozzi, Franco; Carreira, Patricia E.; Bancel, Dominique Farge; Allanore, Yannick; Mueller-Ladner, Ulf and Distler, Oliver, et al. (2010) In Annals of the Rheumatic Diseases 69(10). p.1809-1815
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... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Annals of the Rheumatic Diseases
volume
69
issue
10
pages
1809 - 1815
publisher
British Medical Association
external identifiers
  • wos:000282006800014
  • scopus:77957286169
ISSN
1468-2060
DOI
10.1136/ard.2009.114264
language
English
LU publication?
yes
id
f4a25a54-4299-4d39-bbc2-2a566834ad48 (old id 1696109)
date added to LUP
2010-10-25 12:27:19
date last changed
2018-07-15 04:03:27
@article{f4a25a54-4299-4d39-bbc2-2a566834ad48,
  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.},
  author       = {Tyndall, Anthony J. and Bannert, Bettina and Vonk, Madelon and Airo, Paolo and Cozzi, Franco and Carreira, Patricia E. and Bancel, Dominique Farge and Allanore, Yannick and Mueller-Ladner, Ulf and Distler, Oliver and Iannone, Florenzo and Pellerito, Raffaele and Pileckyte, Margarita and Miniati, Irene and Ananieva, Lidia and Gurman, Alexandra Balbir and Damjanov, Nemanja and Mueller, Adelheid and Valentini, Gabriele and Riemekasten, Gabriela and Tikly, Mohammed and Hummers, Laura and Henriques, Maria J. S. and Caramaschi, Paola and Scheja, Agneta and Rozman, Blaz and Ton, Evelien and Kumanovics, Gabor and Coleiro, Bernard and Feierl, Eva and Szucs, Gabriella and Von Muehlen, Carlos Alberto and Riccieri, Valeria and Novak, Srdan and Chizzolini, Carlo and Kotulska, Anna and Denton, Christopher and Coelho, Paulo C. and Koetter, Ina and Simsek, Ismail and de la Pena Lefebvre, Paloma Garcia and Hachulla, Eric and Seibold, James R. and Rednic, Simona and Stork, Jiri and Morovic-Vergles, Jadranka and Walker, Ulrich A.},
  issn         = {1468-2060},
  language     = {eng},
  number       = {10},
  pages        = {1809--1815},
  publisher    = {British Medical Association},
  series       = {Annals of the Rheumatic Diseases},
  title        = {Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database},
  url          = {http://dx.doi.org/10.1136/ard.2009.114264},
  volume       = {69},
  year         = {2010},
}