Mortality in patients with Sjögren disease: a prospective cohort study identifying key predictors

Abstract

Objective: We aimed to quantify the mortality risk in a large, well-characterized cohort of patients with Sjögren disease (SjD) 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 Prospective cohort. Detailed data on systemic manifestations, serological markers, disease activity, and mortality were collected after a median of 9.5 (IQR 9.2-9.9) years of follow-up. The primary outcome was overall mortality, and 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 of 1.7. Infections (35.7%), malignancies (23.8%), and cardiovascular disease (CVD; 7.1%) were the most common causes of death. Multivariate analysis revealed that older age (HR 1.11/year, 95% CI 1.07-1.15), C4 hypocomplementemia (HR 3.75, 95% CI 1.55-9.06), elevated erythrocyte sedimentation rate (ESR; HR 1.01, 95% CI 1.00-1.03), history of heart failure (HR 4.24, 95% CI 1.02-17.58), and pulmonary involvement (HR 3.31, 95% CI 1.39-7.88) were independent predictors of mortality. Conclusion: This study found a significantly increased mortality risk in SjD, with infections, malignancies, and CVD 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.