Ten-year survival analysis of patients with primary Sjögren's syndrome in China: a national prospective cohort study.

Aims To investigate the long-term survival of patients with primary Sjogren's syndrome (pSS) in China. Methods Patients with pSS who fulfilled the 2002 American-European Consensus Group classification criteria were prospectively enrolled from 2004 to 2011. Their baseline clinical, laboratory, and therapeutic data were collected. The primary endpoint was all-cause death by January 2018. The standard mortality ratio (SMR) was calculated by comparing with age-matched and sex-matched mortality data of the general population. Kaplan-Meier curves were obtained by time-to-event analysis. Univariate and multivariate Cox hazards regression analyses were performed to identify risk factors for mortality. Results A total of 1054 patients were enrolled and 834 patients were followed up for a median of 94.8 months, with 48 confirmed deaths. The total SMR was 3.63 [95% confidence interval (CI) 2.60-4.66]. The 3-, 5-, and 10-year survival rates were 98.4%, 97.5%, and 92.9%, respectively. Infection, malignancy, and respiratory failure were the top three causes of mortality. We identified male sex [hazard ratio (HR) = 3.00, 95% CI 1.23-7.31], age at diagnosis ⩾50 years of age (HR = 7.69, 95% CI 3.01-19.62), thrombocytopenia (HR = 1.93, 95% CI 1.01-3.72), and interstitial lung disease (HR = 5.96, 95% CI 2.24-15.82) as the independent prognostic factors of death. Conclusions The mortality rates of Chinese patients with pSS are higher than those of the general population. Male patients of elder age at diagnosis complicated with thrombocytopenia and interstitial lung disease might be suggestive for poorer survival and require closer follow up.