Background Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease with unknown aetiology and involves mainly salivary and lacrimal glands. It is an autoimmune disease with a broad range of systemic manifestations. Mortality rate might reduce in pSS patients with treated new medications. There is no information on survival of pSS patients in our country.
Objectives This study was conducted to determine clinical features and survival in pSS patients
Methods All pSS patients who diagnosed betwen 2004–2014 years were included in this study. The clinical and laboratory features and mortality rates were reevaluated
Results There were 718 patients with suspected pSS at 10 years. 372 patients were classified as pSS according to 2012 American Collage of Rheumatology Classification Criteria for Sjögren's Syndrome. Women/men ratio was 11/1. The mean age at the time of diagnosis was 50.3±11.81 years. 14 patients (3.8%) were diagnosed different malignancy and 18 patients died (4.8%). The rate of 10-year survival was 95.6% (%96.6 for women, %86.6 for men, p:0.004). 10-year survival rate was % 98.7 in patients without pulmonary involvement, but 79.2% in patients with pulmonary involvement. Multivariate Cox analysis showed that pulmonary involvement (OR:12.8, %95 CI: 4.52–44.8, p<0.001) and male gender (OR:1.7%95, CI: 1.17–4.53, p=0.004) were independent mortality risk factors
Conclusions Our data revealed that cumulative survival was 95.6% at 10 years in pSS patients. Pulmonary involvement represented the main cause of death. pSS patients with pulmoner involvement have greater risk of death.
Disclosure of Interest None declared