Immunoglobulin G4-Related Pancreatic and Biliary Diseases

BACKGROUND: Autoimmune pancreatitis and autoimmune cholangitis are new clinical entities that are now recognized as the pancreaticobiliary manifestations of immunoglobulin (Ig) G4-related disease.

OBJECTIVE: To summarize important clinical aspects of IgG4-related pancreatic and biliary diseases, and to review the role of IgG4 in the diagnosis of autoimmune pancreatitis (AIP) and autoimmune cholangitis (AIC).

METHODS: A narrative review was performed using the PubMed database and the following keywords: “IgG4”, “IgG4 related disease”, “autoimmune pancreatitis”, “sclerosing cholangitis” and “autoimmune cholangitis”. A total of 955 articles were retrieved; of these, 381 contained relevant data regarding the IgG4 molecule, pathogenesis of IgG-related diseases, and diagnosis, management and long-term follow-up for patients with AIP and AIC. Of these 381 articles, 66 of the most pertinent were selected.

RESULTS: The selected studies demonstrated the increasing clinical importance of both AIP and AIC, which can mimic pancreatic cancer and cholangiocarcinoma, respectively. IgG4 titration in tissue or blood cannot be used alone to diagnose all IgG4-related diseases; however, it is often a useful adjunct to clinical, radiological and histological features. AIP and AIC respond to steroids; however, relapse is common and long-term maintenance treatment often required.

CONCLUSIONS: A review of the diagnosis and management of both AIC and AIP is timely and pertinent to clinical practice because the amount of information regarding these conditions has increased substantially in the past few years, resulting in significant impact on the clinical management of affected patients.