IgG4-related disease

Abstract

Immunoglobulin G subclass 4-related disease (IgG4-RD) is a relatively recently identified immune-mediated systemic disease characterised by inflammation and progressive fibrosis. The most common clinical manifestations are pancreatitis and sialadenitis, but the disease may affect virtually any organ in the body. Microscopic examination of biopsy specimens reveals inflammation with a substantial proportion of IgG4-positive plasma cells and a characteristic fibrosis pattern. Glucocorticoids are the first-line choice of treatment.