Survival and death causes in 251 systemic sclerosis patients from a single Italian center

Objective: To investigate survival in Italian systemic sclerosis (SSc) patients from a tertiary center, reporting death causes. Materials and methods: We analyzed the charts of 251 SSc patients prospectively enrolled in our Rheumatology Unit from 2000 to 2008. Baseline characteristics were recorded. In 2008 the vital status and the causes of death were assessed. Overall and subgroup survival were analyzed by the Kaplan-Meier method and the log-rank test. Results: In 2008, 82% of patients were alive, 8% were known to have died and 10% were lost to follow-up. Overall 5- and 8-year survival were 94.8% and 77.1%, respectively. Patients with an age greater than the median value of the cohort (χ2=4.4; p=0.036), diffuse cutaneous SSc (χ2=3.9; p=0.048), digital ulcers (χ2=6; p=0.015), articular (χ2=5.3; p=0.021), lung (χ2=5.6; p=0.018) and heart involvement (χ2=9.3; p=0.002) had a poorer survival than patients without these features. The majority of SSc-related deaths (60%) were secondary to interstitial lung disease and heart involvement (both 33.3%); 50% of non-SSc-related deaths were due to cancer. Conclusions: Our study reports an improvement in survival of Italian SSc patients during the last decade with respect to the previous ones. Moreover, a reduction in deaths from renal involvement and an increase in deaths from interstitial lung disease were recorded in Italian SSc patients. Our data are consistent with those from recent survival studies carried out on SSc patients from other geographic areas.