Background Patients (pts) with systemic sclerosis (SSc) experience higher mortality rates.
Objectives To assess the mortality and causes of death in SSc pts' cohort at our center.
Methods The data were retracted from the Rambam EUSTAR database (220 pts, years 2004–2013) and completed from pts' files; demographic, clinical and laboratory data, malignancy cases, the reason of death were analyzed. Comparisons were made between the deceased pts and surviving pts with similar disease duration. Proportional hazards models of Kaplan-Meier survival analysis and student's T-test were used for statistical analysis. Data found to significantly affect survival in a first univariate analysis were entered in a multivariate Cox proportional hazards model. Causes of death were analyzed using standard descriptive statistics. Data characterized pts with malignancy were incorporated in the logistic regression model.
Results 47 (21.4%) pts deceased (age 53±15 years, disease duration 9.8±11 years, females 76.6%, diffuse disease 27.6%). Causes of death were: pulmonary fibrosis (PF), pulmonary arterial hypertension (PAH), right and left heart failure (CHF, 27.7% and 19.1%); infections (17.0%), malignancies (17.0%), renal crisis (6.4%), gastrointestinal complications (GIT) (2.1%), and others (10.64%). Survival was significantly affected by: older age at diagnosis (P=0.011), male gender (P=0.049), reduced lung volumes and diffusing capacity (P=0.014 and p=0.001), PF (P=0.002), PAH (P<0.001), CHF (P<0.001), use of diuretics (P<0.001), arrhythmias (P<0.001), malignancy (P=0.003), proteinuria (P=0.003), and GIT complications (P=0.027). Malignancy was reported in 14/47 (29.8%) pts: lungs 23.5%, GIT 17.7%, genitourinary tract 17.7%, other 41.2%. Malignancy was not an independent predictor of mortality.
Conclusions In this study the leading cause of death in SSc pts was cardiopulmonary complications, followed by malignancy and infections; renal and GIT complications were less frequent. High index of suspicion and early diagnosis of main SSc complications will allow early intervention and may hopefully change the fate of SSc pts.
Disclosure of Interest None declared