Immunoglobulin G4-related cholangitis

Immunoglobulin G4-related cholangitis

This recently recognised entity presents with di ff use or segmental narrowing of the intra- or extrahepatic bile ducts. Its features may make di ff erentiation from PSC, cholangiocarci noma and pancreatic cancer di ffi cult. However, patients often have elevated serum IgG4 levels and concomitant autoimmune pancreatitis, IgG4-related sialadenitis or retroperitoneal fibr sis. Biliary biopsies show lymphoplasmacytic sclerosing cholan gitis. Treatment is with systemic steroids. Failure to respond to steroid therapy should make one reconsider the diagnosis and exclude an underlying malignancy .