S3480 Autoimmune Hepatitis and IgG4 Cholangiopathy Overlap in an Inflammatory Bowel Disease Patient

Abstract

INTRODUCTION: Extraluminal manifestations of inflammatory bowel disease (IBD) are well documented, however, hepatic manifestations such as autoimmune hepatitis (AIH) and IgG4 cholangiopathy (IAC) are rare. CASE DESCRIPTION/METHODS: We describe a case of a 32-year-old Hispanic man with ulcerative pancolitis refractory to vedolizumab therapy who developed abnormal liver tests with normal hepatobiliary imaging but histologic and serologic markers consistent with AIH and IgG4 cholangiopathy overlap. His hepatobiliary disease was responsive to steroids and subsequently maintained on immunomodulator therapy. DISCUSSION: Proper diagnosis and treatment of hepatobiliary abnormalities can be challenging, particularly, when it involves overlapping diseases like IBD, AIH, and IAC. IAC is commonly misdiagnosed as PSC. This case highlights the diagnostic dilemma and management of a rare combination of diseases in an IBD patient.