title: Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.
authors.affiliation: Department of Rheumatology, University of Basel, Basel, Switzerland.
authors.forename: Anthony J
authors.initials: AJ
authors.lastname: Tyndall
authors.forename: Bettina
authors.initials: B
authors.lastname: Bannert
authors.forename: Madelon
authors.initials: M
authors.lastname: Vonk
authors.forename: Paolo
authors.initials: P
authors.lastname: Airò
authors.forename: Franco
authors.initials: F
authors.lastname: Cozzi
authors.forename: Patricia E
authors.initials: PE
authors.lastname: Carreira
authors.forename: Dominique Farge
authors.initials: DF
authors.lastname: Bancel
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
authors.forename: Ulf
authors.initials: U
authors.lastname: Müller-Ladner
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.forename: Florenzo
authors.initials: F
authors.lastname: Iannone
authors.forename: Raffaele
authors.initials: R
authors.lastname: Pellerito
authors.forename: Margarita
authors.initials: M
authors.lastname: Pileckyte
authors.forename: Irene
authors.initials: I
authors.lastname: Miniati
authors.forename: Lidia
authors.initials: L
authors.lastname: Ananieva
authors.forename: Alexandra Balbir
authors.initials: AB
authors.lastname: Gurman
authors.forename: Nemanja
authors.initials: N
authors.lastname: Damjanov
authors.forename: Adelheid
authors.initials: A
authors.lastname: Mueller
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.forename: Gabriela
authors.initials: G
authors.lastname: Riemekasten
authors.forename: Mohammed
authors.initials: M
authors.lastname: Tikly
authors.forename: Laura
authors.initials: L
authors.lastname: Hummers
authors.forename: Maria J S
authors.initials: MJ
authors.lastname: Henriques
authors.forename: Paola
authors.initials: P
authors.lastname: Caramaschi
authors.forename: Agneta
authors.initials: A
authors.lastname: Scheja
authors.forename: Blaz
authors.initials: B
authors.lastname: Rozman
authors.forename: Evelien
authors.initials: E
authors.lastname: Ton
authors.forename: Gábor
authors.initials: G
authors.lastname: Kumánovics
authors.forename: Bernard
authors.initials: B
authors.lastname: Coleiro
authors.forename: Eva
authors.initials: E
authors.lastname: Feierl
authors.forename: Gabriella
authors.initials: G
authors.lastname: Szucs
authors.forename: Carlos Alberto
authors.initials: CA
authors.lastname: Von Mühlen
authors.forename: Valeria
authors.initials: V
authors.lastname: Riccieri
authors.forename: Srdan
authors.initials: S
authors.lastname: Novak
authors.forename: Carlo
authors.initials: C
authors.lastname: Chizzolini
authors.forename: Anna
authors.initials: A
authors.lastname: Kotulska
authors.forename: Christopher
authors.initials: C
authors.lastname: Denton
authors.forename: Paulo C
authors.initials: PC
authors.lastname: Coelho
authors.forename: Ina
authors.initials: I
authors.lastname: Kötter
authors.forename: Ismail
authors.initials: I
authors.lastname: Simsek
authors.forename: Paloma García
authors.initials: PG
authors.lastname: de la Pena Lefebvre
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: James R
authors.initials: JR
authors.lastname: Seibold
authors.forename: Simona
authors.initials: S
authors.lastname: Rednic
authors.forename: Jirí
authors.initials: J
authors.lastname: Stork
authors.forename: Jadranka
authors.initials: J
authors.lastname: Morovic-Vergles
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
mesh_terms: D000328:Adult; D000368:Aged; D015897:Comorbidity; D004812:Epidemiologic Methods; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000401:mortality; D006331:Heart Diseases / Q000401:mortality; D006801:Humans; D008171:Lung Diseases / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000401:mortality; D011014:Pneumonia / Q000401:mortality; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000401:mortality*; D018805:Sepsis / Q000401:mortality