Risk factors for death and the 3-year survival of patients with systemic sclerosis: the French ItinérAIR-Sclérodermie study.
OBJECTIVE
This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline.


METHODS
At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier. Survival was estimated using the Kaplan-Meier method. Multivariate survival analyses were conducted using the Cox model.


RESULTS
In total, 546 patients were followed for a median duration of 37 months, representing 1547 patient-years. At baseline, the majority of patients were female, with lcSSc, mean age 54.9 +/- 13.0 years and mean duration of SSc of 8.8 +/- 8.1 years. In total, 47 patients died, giving a 3-year survival of 91.1% and cumulative mortality of 3.04 deaths per 100 patient-years; 17 deaths (32.2%) resulted from pulmonary arterial hypertension (PAH) and eight (17.1%) from cancer. Of the 47 patients with PAH at baseline, 20 died during follow-up, giving a 3-year survival of 56.3%. In a multivariate analysis, PAH [hazard ratio (HR) 7.246], age at first symptom (HR 1.052), duration of SSc (HR 1.047 per year) and Rodnan skin score (per one point) (HR 1.045) were associated with increased mortality.


CONCLUSIONS
This 3-year study observed survival and mortality estimates that were comparable with previous reports. PAH increased the HR for mortality in patients with SSc, justifying yearly echocardiographic screening.
Hachulla
Eric
E
Department of Internal Medicine, National Reference Center for Scleroderma, Claude Huriez Hospital, University of Lille, 59037 Lille cedex, France. ehachulla@chru-lille.fr
Carpentier
Patrick
P
Gressin
Virginie
V
Diot
Elisabeth
E
Allanore
Yannick
Y
Sibilia
Jean
J
Launay
David
D
Mouthon
Luc
L
Jego
Patrick
P
Cabane
Jean
J
de Groote
Pascal
P
Chabrol
Amélie
A
Lazareth
Isabelle
I
Guillevin
Loïc
L
Clerson
Pierre
P
Humbert
Marc
M
D000328:Adult; D017668:Age of Onset; D000368:Aged; D004812:Epidemiologic Methods; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications / Q000401:mortality*; D013997:Time Factors