Hachulla, E., Carpentier, P., Gressin, V., Diot, E., Allanore, Y., Sibilia, J., Launay, D., Mouthon, L., Jego, P., Cabane, J., de Groote, P., Chabrol, A., Lazareth, I., Guillevin, L., Clerson, P., Humbert, M., the ItinerAIR-Sclerodermie Study Investigator
Objectives. 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.
Conclusion. 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.