IgG4 Associated Cholangitis and Hepatic Pseudoinflammatory Tumour: IgG4 Related Disease Mistaken by Neoplastic Disease

Abstract

inaugural cholestatic jaundice, with a stricture and circumferential wall thickening of the distal common bile duct, as well as an ill-defined hepatic infiltrative mass with “amputation” of peripheral distended bile ducts. The patient underwent a pancreaticoduodenectomy and hemihepatectomy for suspected cholangiocarcinoma. Histology revealed IgG4 associated cholangitis (IgG4-AC) and hepatic pseudoinflammatory tumour (HPT).
IgG4-related disease (IgG4-RD) is a recently established systemic disease, characterized by fibro-inflammatory tissue infiltrates and tumefaction of one or more organs. The hepatobiliary manifestations comprise IgG4-AC and associated HTP - which can be mistaken for cholangiocarcinoma. Clinicians must be familiar with this disorder, usually responsive to corticosteroid therapy, in order to avoid unnecessary invasive procedures.
We present a case of IgG4-AC and associated HTP and review the literature of this rare presentation of IgG4-RD.