POS1296 SURVIVAL RATES IN PATIENTS WITH SYSTEMIC SCLEROSIS ARE AFFECTED BY CONCURRENT CARDIOPULMONARY DISEASE, GENDER, AND AUTOANTIBODY PROFILE: A 15- YEAR SINGLE CENTER EXPIERENCE

Abstract

BackgroundSystemic Sclerosis (SSc) is a systemic autoimmune disease with a high mortality rate[1-3].ObjectivesThe purpose of this study was to assess long-term survival rates of patients with SSc in a single rheumatology center and identify factors affecting the survival rates.MethodsRetrospective analysis of unselected adult patients with SSc, diagnosed and followed up in the Rheumatology Clinic of the University Hospital of Larissa in Central Greece for 15 years (2007-2022). Demographics and clinical characteristics were retrieved from medical records. while autoantibodies (autoAbs) were assessed by indirect immunofluorescence and a SSc autoantibody line blot immunoassay (Euroimmun). Overall Survival (OS), 5-year and 10-year survival rates were calculated. The effect of age at disease onset, gender, clinical parameters, and autoantibodies were evaluated using Kaplan-Meier analysis.ResultsA total of 226 patients (85.8% female, mean age 61.9 years) were included. The mean ± SD duration of the disease was 12.24±8.9 years. The 5-year and 10-year survival were 95.59%, and 88.1%, respectively. In the majority of patients, the cause of death was related to SSc. The two main causes of death were interstitial lung disease (ILD) and heart disease (pulmonary arterial hypertension and/or arrythmias). Older age at disease onset was independently associated with worse survival (58.9± 11.9 years vs 47.3±14.6 years, p<0.05) and male patients had statistically worse OS compared to female patients (13.1 years vs 28.4 years, p=0,001). Patients with autoAbs against Th/To (12.1 years vs 28.2 years, p=0.013) or against NOR90 had worse OS compared to autoAb-negative patients (12.1 vs 28.2 years, p=0.013, and 12.7 vs 28.8 years p=0.007, respectively). There was a trend for a better survival in patients with anti-centromere autoAbs (29.4 vs 26.2 years, p=0.064).ConclusionOur results show that the overall survival rates of patients with SSc have improved over the years than those reported in the past. Cardiopulmonary diseases still remain the main causes of death, while male sex is associated with worse prognosis. Additional prognostic factors have emerged from autoAb profiles.