Central nervous system disease in primary Sjögren's syndrome

Primary Sjogren's syndrome is a systemic autoimmune disorder whose main characteristics are dryness of the eyes and mouth, caused by lymphocytic infiltration of the exocrine glands. Patients may also show signs of extraglandular involvement of lung, liver, kidney and vessel walls, as well as of central and peripheral nervous system, muscles and joints. We present a review of the literature on the involvement of the central nervous system in primary Sjögren's syndrome, which may mimic many other neurologic disorders, especially multiple sclerosis. Features that may help in distinguishing these two entities are presented.