Extraglandular Manifestations of Primary Sjogren's Syndrome

Sjogren's syndrome is a chronic autoimmune disease that typically affects the salivary and lacrimal glands. Aside from the common glandular signs and symptoms, Sjogren's syndrome may also cause mononuclear infiltration and immune complex deposition involving extraglandular sites producing several extraglandular manifestations (EGM). The prevalence of EGMs varies greatly depending on the particular manifestation. This article examines the ways that EGMs may present in patients with primary Sjogren's syndrome. The focus is on the more prevalent and significant EGMs including involvement of the nervous system, pulmonary manifestations, vasculitis associated with primary Sjogren's syndrome, and arthropathy.