IgG4-Related Disease.

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is a multisystem disorder characterized histologically by an IgG4-positive lymphoplasmacytic tissue infiltrate, storiform fibrosis and an obliterative phlebitis. The HISORt criteria can be used to establish the diagnosis and incorporate a multidisciplinary approach, involving histology, radiology, serum IgG4 concentrations and response to corticosteroid therapy. IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the disease, which often occurs in association with autoimmune pancreatitis (AIP).