Higher Mortality Risk From Ovarian Carcinomas, Small Bowel Neoplasms, and B-Cell and Mucosa-Associated Lymphoid Tissue Lymphomas in Sjögren Syndrome Patients.

Objective: To evaluate the impact of the different types of neoplasms and lineages on Sjögren syndrome (SjS) patient mortality.

Methods: Medical records review study based on the Spanish Hospital Discharge Database and the International Classification of Diseases, Tenth Revision, Clinical Modification coding list. The neoplasm-related deaths in SjS patients with the general population during the period 2016-2019 were compared. A binary logistic regression analysis considering age, sex, tobacco use, and alcohol use was performed to determine the impact of SjS on the risk of dying from each neoplasm group and lineage.

Results: In the period studied, 705,557 in-hospital deaths were certified in Spain, 139,531 (19.8%) from neoplasms. Neoplasms surpassed SjS activity as a cause of mortality in primary SjS patients (11.3% vs. 1.6%, p < 0.001). SjS patients presented higher mortality rates from small bowel carcinoma (0.3% vs. 1.8%; odds ratio [OR], 5.41; 95% confidence interval [CI], 1.33-22) and gynecological neoplasms (6.4% vs. 3%; OR, 2.13; 95% CI, 1.01-4.58), related to ovarian carcinomas (4.6% vs. 1.3%; OR, 3.65; 95% CI, 1.48-8.97), than the general population. Hematological neoplasm-related deaths were more prevalent in SjS patients than in the non-SjS population (18.3% vs. 8.9%; OR, 2.04; 95% CI, 1.25-3.31), mostly attributable to the higher proportion of deaths from B-cell non-Hodgkin lymphoma (8.3% vs. 2.5%; OR, 3.04; 95% CI, 1.54-6.03) and mucosa-associated lymphoid tissue lymphoma (1.8% vs. 0.1%; OR, 70.17; 95% CI, 16.61-296.36).

Conclusion: SjS patients face an elevated risk of mortality from small bowel neoplasms, ovarian carcinomas, and B-cell and mucosa-associated lymphoid tissue lymphoma compared with the general Spanish population. Apart from developing approaches to mitigate their occurrence, it is crucial to explore thoroughly and consider the implementation of targeted early-detection programs for these specific conditions.