Research Article Clinical Course, Prognosis, and Cause of Death in Primary Sjögren’s Syndrome

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The aim of this retrospective, single-centre study was to investigate the clinical and laboratory features and disease outcomes of 547 patients diagnosedwith primary Sjögren’s syndrome (pSS) between 1975 and 2010.The patients were followed up for 11.4±6.2 years. We evaluated the clinical and laboratory features, and assessed their influence on the time of diagnosis, survival, andmortality ratios, and compared themwithin subgroups defined by gender, glandular and extraglandularmanifestations (EGMs), associated diseases, and immunoserological abnormalities.Themost frequent EGMs were polyarthritis, Raynaud’s phenomenon, and vasculitis among our patients; themost common associated diseasewas thyroiditis. During the follow-up period, 51 patients died; themedian survival time was 33.71 years. Our results revealed a negative effect of cryoglobulinemia on survival ratios; additionally, the presence of vasculitis and lymphoproliferative diseases at the time of diagnosis increased the risk of mortality. The development of vasculitis was themost powerful predictor ofmortality.Mortality in the group of patients with extraglandular symptomswas two- to threefold higher than in the glandular group. Attention is drawn to the importance of close monitoring and targeted diagnostic approaches in those pSS subgroups with obviously increased mortality risk. 1