Overview of Sjögren syndrome

Sjögren syndrome is the most common inflammatory connective tissue disease after rheumatoid arthritis (RA) and affects 0.3 to 3% of the population. Sjögren syndrome may be primary (idiopathic) or secondary, when it occurs in association with RA or other inflammatory connective tissue disease, such as systemic lupus erythematosus (SLE) or systemic sclerosis. Primary Sjögren syndrome predominantly affects females and the usual age of onset is between 40 and 50 years. The main features of Sjögren syndrome are severe sicca symptoms (see Clinical features of Sjögren syndrome), but other features common to inflammatory connective tissue diseases may also be present.

An important differential diagnosis for Sjögren syndrome is immunoglobulin G4–related disease (IgG₄), which may present with significant sicca symptoms.

Immunomodulatory drugs (eg methotrexate, azathioprine, cyclophosphamide, rituximab) may be used for severe refractory disease; see Management for Sjögren syndrome.