[Central nervous system involvement in primary Sjögren syndrome]

Abstract

Sjögren syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands and presents with sicca symptoms of the main mucosa surfaces. The spectrum of the disease extends from sicca syndrome to systemic involvement (extraglandular manifestations). Although earlier studies described central nervous system (CNS) involvement as a frequent extraglandular manifestation of primary SS, symptomatic CNS involvement is rarely described in recent large series (<5%). A wide spectrum of neurological features has been described, including not only asymptomatic white matter lesions in MR examinations, but also severe focal or diffuse neurological processes. Differential diagnosis includes the coexistence of other systemic autoimmune diseases (lupus, antiphospholipid syndrome, vasculitis), cerebrovascular disease and multiple sclerosis.