Nervous system involvement in primary Sjögren’s syndrome: analysis from the Sjögrenser registry
Ximena Larco-Rojas, Mónica Fernández Castro, Carlos Sánchez-Piedra, Víctor Martínez Taboada, Alejandro Olivé, José Rosas, Raúl Menor, Beatriz Rodríguez, Ángel García-Aparicio, Francisco Javier López Longo, Sara Manrique- Arija, Jesús Alberto García Vadillo, Susana Gil Barato, Ruth López Gonzalez, Francisco Javier Narváez, Carlos Galisteo, Jorge Gonzalez Martín, Esther Ruiz Lucea, Antonio Naranjo, Óscar Illera, Sheila Melchor, Begoña Moreira, Enrique Raya, José Luis Puga Guzmán, Natalia Cid, Enrique Júdez, Clara Moriano, Vicente Torrente-Segarra, Héctor Corominas, Blanca García Magallón, Carlos Guillén Astete, Ivan Castellvi, Cristina Bohórquez, Javier Loricera, Nerea Alcorta Lorenzo, Jose Luis AndreuAbstract
Objective
This study aims to describe the clinical characteristics of nervous system involvement in a cohort of patients from the SjögrenSER registry.
Methods
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.
Results
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).
Conclusions
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.