Background Systemic sclerosis (SSc) is associated with a significant reduction in survival in comparison to the general population. This is the first report on 5-year survival and its predictors in a Romanian cohort of SSc patients.
Objective We aimed to assess the 5-year survival rate in a single-center cohort of SSc patients and to identify predictors of death and disease worsening.
Methods All patients of the EUSTAR Center 100 enrolled before 2009 and who had at least 2 visits at a minimum interval of 5 years, or who died after at least 3 months of follow-up, were included. All patients were assessed according to current EUSTAR recommendations. A comparison was made between the surviving and the deceased patients regarding all MEDS baseline parameters. Using age-adjusted univariate logistic regression we identified predictors for death and for several outcomes considered as disease worsening: 20% reduction in forced vital capacity (FVC); 20% reduction in diffusing capacity of the lung for carbon monoxide (DLCO); development of pulmonary arterial hypertension (PAH) as assessed by power Doppler heart ultrasound; and digital ulcers (DUs) recurrent at prospective visits.
Results Out of 68 patients enrolled before 01.01.2009, 40 met the inclusion criteria (82,5% females, 55% limited cutaneous subset, mean ± SD follow-up period 5.7 ± 2.1 years; mean ± SD age at first visit 49 ± 11.8 years; mean ± SD disease duration at first visit 4.4 ± 6.0 years). Throughout the 5-year follow-up period there were 7 deaths (including 3 SSc-related deaths), resulting in an overall 5-year survival rate of 82,5%. In survivors, lung function tests deteriorated significantly in 11% (FVC) and 52% (DLCO), while 20% developed PAH. Recurrent or newly appearing DUs occurred in 21% of all 42 patients. Significant predictors for death were the diffuse cutaneous subset, and the presence of DUs at presentation, with odd ratios [95% confidence interval] (OR[CI95%]) of 14,2 [1.3-151] and 38 [2.9-501] respectively. Conduction blocks on the baseline ECG predicted PAH with an OR [95% CI] of 12.8 [1.5-108]. Significant predictors for DUs were active DUs and calcinosis at presentation: OR [CI95%] 5.2 [1.1-26] and 6 [1.1-33] respectively. None of the parameters tested as potential predictors of the respiratory function decline achieved statistical significance.
Conclusions The survival rate in our cohort was 82.5%, which is similar to other cohorts from developed countries. We identified diffuse cutaneous subset, active digital ulcers, and heart conduction blocks as predictors for a poor disease outcome.