IgG4 Associated Cholangiopathy: Diagnosis, Treatment, and Outcome

Abstract

IgG4-associated cholangitis (IAC) is a subgroup of IgG4-related disease, which is more common in elderly men.IAC is frequently coincident with autoimmune pancreatitis (AIP).However, some IAC cases do not have other organsinvolvement.  The diagnosis of IAC is based on biochemical, radiological, and histological features.Among these, elevated serum levels of IgG4, extra- and intrahepatic biliary strictures (as visualized by cholangiography), lymphoplasmacytic infiltrations in the liver and bile duct tissue, and association with AIP are of key importance. IAC may mimicprimary sclerosing cholangitis or cholangiocarcinoma(CC). It is classically a corticosteroid-responsive condition and corticosteroid is regarded as the initial treatment of choice in this disease.However, relapse following corticosteroid withdrawal is a frequent event.