Biliary and hepatic involvement in IgG4-related disease

IgG4-related disease (IgG4-RD) is a multi-systemic disorder. IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the disease, often in association with autoimmune pancreatitis (AIP). Hepatic manifestations of IgG4-RD are less well described within the literature.

To examine and present an overview of IgG4-RD with a focus on the biliary and hepatic manifestations.

An electronic search using Medline was performed. Search items included 'IgG4 multi-system disease, IgG4 associated cholangitis, IgG4 associated liver disease and autoimmune pancreatitis (AIP)'.

IgG4-RD is characterised by an IgG4-positive lymphoplasmacytic tissue infiltrate, storiform fibrosis and an obliterative phlebitis. The HISORt criteria may be used to establish the diagnosis and incorporate a multi-disciplinary approach involving histology, radiology, serum IgG4 levels and response to steroid therapy. IgG4-SC is the commonest extrapancreatic manifestation of type-1 AIP, while the hepatic manifestations remain poorly defined. Important differential diagnoses include primary sclerosing cholangitis, secondary sclerosing cholangitis, cholangiocarcinoma and pancreatic carcinoma. Current treatment regimens remain ill defined although steroid therapy is used first line unless contraindicated. Patients with relapsing disease or multifocal disease should be considered for azathioprine. Available data would also suggest a role for rituximab.

IgG4-related sclerosing cholangitis is a common manifestation of IgG4-related disease which requires a multi-disciplinary approach to establish the diagnosis. Differentiating IgG4-related sclerosing cholangitis from other conditions, both benign and malignant, is challenging, but vital. Steroids remain the mainstay of treatment. Our understanding of the pathogenesis of the hepatic manifestations of IgG4-related disease continues to evolve.