Hepatobiliary IgG4 Cholangiopathy: Case Series and Literature Review

Abstract

IgG4 cholangiopathy is a recently described distinctive type of hepatobiliary manifestation of IgG4-related disease. This is often misdiagnosed as a malignancy of the hepatobiliary system on clinical presentation and imaging. Accurate diagnosis is crucial for appropriate management. Histopathology provides crucial information and is characterized by IgG4-positive lymphoplasmacytic infiltration with storiform fibrosis and obliterative phlebitis. We present the clinicopathological spectrum of a retrospective series of 6 cases of IgG4 cholangiopathy that clinically masqueraded as hepatobiliary malignancies.

Keywords: IgG4; cholangiopathy; hepatobiliary; obliterative phlebitis; storiform fibrosis.