Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.
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.
Tyndall
Anthony J
AJ
Department of Rheumatology, University of Basel, Basel, Switzerland.
Bannert
Bettina
B
Vonk
Madelon
M
Airò
Paolo
P
Cozzi
Franco
F
Carreira
Patricia E
PE
Bancel
Dominique Farge
DF
Allanore
Yannick
Y
Müller-Ladner
Ulf
U
Distler
Oliver
O
Iannone
Florenzo
F
Pellerito
Raffaele
R
Pileckyte
Margarita
M
Miniati
Irene
I
Ananieva
Lidia
L
Gurman
Alexandra Balbir
AB
Damjanov
Nemanja
N
Mueller
Adelheid
A
Valentini
Gabriele
G
Riemekasten
Gabriela
G
Tikly
Mohammed
M
Hummers
Laura
L
Henriques
Maria J S
MJ
Caramaschi
Paola
P
Scheja
Agneta
A
Rozman
Blaz
B
Ton
Evelien
E
Kumánovics
Gábor
G
Coleiro
Bernard
B
Feierl
Eva
E
Szucs
Gabriella
G
Von Mühlen
Carlos Alberto
CA
Riccieri
Valeria
V
Novak
Srdan
S
Chizzolini
Carlo
C
Kotulska
Anna
A
Denton
Christopher
C
Coelho
Paulo C
PC
Kötter
Ina
I
Simsek
Ismail
I
de la Pena Lefebvre
Paloma García
PG
Hachulla
Eric
E
Seibold
James R
JR
Rednic
Simona
S
Stork
Jirí
J
Morovic-Vergles
Jadranka
J
Walker
Ulrich A
UA
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