Background/Purpose: Primary Sjögren’s syndrome (pSS) can have a systemic involvement with extraglandular manifestations, which are associated with more severe disease and with higher risk of complications, such as lymphoma. pSS has also been associated with an increased prevalence of cardiovascular risk (CV) factors and cardiovascular disease. The aim of the study was to describe the clinical characteristics of a cohort of Spanish patients with pSS from a tertiary hospital and to analyze the rates of CV factors and lymphoma between groups.

Methods: A retrospective descriptive study of patients with a diagnosis of pSS between January 1990 and December 2024 who met the ACR/EULAR 2016 classification criteria for pSS, in follow-up in a tertiary hospital in Madrid, Spain. Patients diagnosed with other systemic connective tissue diseases were excluded. Patients with glandular involvement were compared to patients with systemic involvement (extraglandular). Categorical variables were presented as proportions or percentages, whereas continuous variables were reported as means with standard deviations (SD). Group comparisons were conducted using the Student’s t-test or the χ²test, as appropriate.

Results: A total of 110 patients were included, 39 (35.5%) had extraglandular involvement. Main characteristics are reported in Table 1. There were no differences regarding age, sex, time in follow up and smoking status. Cardiovascular risk (CV) factors were similarly distributed between groups, but a higher proportion of patients with extraglandular involvement had more than 1 CV risk factor. No cardiovascular events were reported after pSS diagnosis. Articular involvement was the most frequent followed by Raynaud, peripheral nervous system and interstitial lung disease. Regarding neoplasia, 14.5% of patients developed malignancies, rates were higher in extraglandular patients. The reported incidence rate of lymphoma was 4.54 per 100 patients with pSS, being 2.82 per 100 patients in the glandular group and 7.69 per 100 patients in the extraglandular group.Hydroxychloroquine was the main treatment in both groups, with higher rates of corticosteroids, rituximab and immunosuppressive treatments in patients with extraglandular involvement, as expected.

Conclusion: Patients with extraglandular involvement in pSS present significantly more severe systemic manifestations with more frequent use of immunosuppressive therapies. They showed a higher cardiovascular risk burden and a considerable prevalence of neoplasia, particularly lymphomas.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/higher-cardiovascular-risk-and-lymphoma-development-in-primary-sjogrens-syndrome-with-extraglandular-involvement/