Nervous system involvement in primary Sjögren's syndrome: analysis from the Sjögrenser registry

This study aims to describe the clinical characteristics of nervous system involvement in a cohort of patients from the SjögrenSER registry.

All patients included in the SjögrenSER study, a Spanish multicenter randomized cohort containing demographic, clinical, and histologic data, were retrospectively analyzed. Patients were classified based on the presence of neurological involvement (central and peripheral nervous systems). We performed descriptive statistical analyses, clinical associations, Student's t test or χ2 test, and uni- and multivariate logistic regression.

The registry included 437 patients, with peripheral nervous system involvement observed in 39 patients (8.9%) and central nervous system involvement in 34 patients (7.8%). Stroke and ischemic cardiovascular disease were common comorbidities in patients with Sjögren's disease (SjD) and neurological involvement (p < 0.05). Neurological involvement was also frequently associated with other systemic manifestations, including renal involvement (p = 0.015), particularly glomerulonephritis with cryoglobulinemia (p = 0.021), and myositis (p = 0.002). Fatigue was significantly associated with neurological involvement (p = 0.012).Patients with nervous system involvement more frequently received glucocorticoids, antimalarials, immunosuppressants, intravenous immunoglobulins, and biologic therapies. Notably, steroids and cyclophosphamide were used more often than other medications (OR = 3.33; 1.54-7.18 and OR = 6.80; 1.49-30.96, respectively).

In this cohort, neurological involvement was commonly associated with other systemic manifestations of SjD, such as myositis, suggesting a more severe disease phenotype that often requires immunosuppressants and biologic therapy.