IgG4-associated cholangitis.

IgG4-associated cholangitis is the hepatobiliary manifestation of a recently characterized inflammatory systemic disease, associated with increased IgG4 serum levels and IgG4-positive lymphoplasmacytic infiltration. Often, patients present with obstructive jaundice, and imaging reveals stenoses of the extrahepatic or intrahepatic bile ducts, often in association with parenchymal pancreatic findings and irregularities of the pancreatic duct. The histologic findings include lymphoplasmacytic infiltrates, on occasion resulting in tumefactive lesions (which can mimic malignancy), obliterative phlebitis, and fibrotic changes. Steroid treatment is the mainstay of management, but relapse is common after discontinuation of therapy or during tapering of steroids and may require further treatment.