Mortality in patients with Sjögren Disease: A Prospective Cohort Study Identifying Key Predictors.

Abstract

OBJECTIVE To quantify the mortality risk in a large, well-characterized cohort of Sjögren's disease (SjD) patients and to identify independent predictors of mortality in this population. METHODS We included 314 patients diagnosed with SjD according to the 2002 American-European Consensus Group criteria from a prospective, multicenter SjögrenSER-PROS cohort. Detailed data on systemic manifestations, serological markers, disease activity, and mortality was collected after 9 years of follow up. The primary outcome was overall mortality, secondary analyses aimed to identify independent predictors of mortality using Cox proportional hazards models. Standardized mortality ratios were calculated by comparing the observed deaths in the SjD cohort to the expected deaths in an age- and sex-matched general population. RESULTS The study identified a 70% increased mortality risk in the SjD cohort compared to the general population, with a standard mortality ratio (SMR) of 1.7. Infections (35.71%), malignancies (23.8%), and cardiovascular disease (7.14%) were the most common causes of death. Multivariate analysis revealed that older age (HR 1.11 per year, 95% CI 1.05-1.17), C4 hypocomplementemia (HR 3.75, 95% CI 1.52-9.24), elevated erythrocyte sedimentation rate (HR 1.01, 95% CI 1.00-1.03), history of heart failure (HR 4.24, 95% CI 1.89-9.51), and pulmonary involvement (HR 3.31, 95% CI 1.48-7.41) were independent predictors of mortality. CONCLUSION This study shows a significantly increased mortality risk in SjD, with infections, malignancies, and cardiovascular disease as leading causes of death. Independent predictors of mortality include advanced age, C4 hypocomplementemia, elevated ESR, heart failure, and pulmonary involvement, underscoring the need for proactive, individualized management.