Diagnosis and management of central nervous system Sjögren’s syndrome

Central nervous system (CNS) involvement is a relatively uncommon manifestation of Sjögren’s syndrome (SS) compared with other extraglandular features. However, it may include a wide spectrum of clinical syndromes ranging from asymptomatic white matter disease to vasculitis, to incapacitating demyelinating syndromes such as optic neuritis and transverse myelitis. Central nervous system disease may be the presenting manifestation of Sjögren’s syndrome and may occur in patients with mild, glandular disease in the absence of other systemic manifestations, or in those with active systemic disease. Therefore a high index of suspicion is required to diagnose and treat these manifestations in a timely manner. This is particularly important, as many neurologic syndromes can present acutely or be rapidly progressive, leading to irreversible neurologic disability. Early institution of treatment is necessary to mitigate the effects of these syndromes, and can have a significant impact on patients’ overall morbidity and mortality.