Unusual Presentation of Autoimmune Hepatitis in Pre-Existing Ulcerative Colitis

Abstract

Purpose: About 16% of patients with Autoimmune hepatitis (AIH) have ulcerative colitis (UC). Conventionally, AIH is characterized by antinuclear antibodies (ANA), anti smooth muscle antibodies (ASMA), and/or anti liverkidney microsmal antibodies (anti-LKM-1). We present three cases from a single institution of newly diagnosed AIH in UC patients with negative ANA, ASMA, and anti-LKM-1. Case 1: A 56-year-old Chinese male with a 22 year history of UC well controlled on mesalamine presents with three weeks of dark urine accompanied by lethargy and two weeks of jaundice. His AST and ALT were both over 2000, with an elevated gamma globulin fraction. His additional liver workup was negative. His ANA, ASMA, and Anti-LKM-1 were negative. The MRCP was negative for PSC. The liver biopsy showed markedly active chronic hepatitis with confluent necrosis and a predominance of plasma cells. pANCA was strongly positive. His liver function normalized with initiation of prednisone and 6-mercaptopurine (6-MP). Case 2: A 20-year-old Hispanic male with an eight year history of UC well controlled on mesalamine was admitted to the hospital with jaundice and right upper quadrant pain. His AST and ALT were over 700, with an elevated gamma globulin fraction. His additional liver workup was negative. His ANA, ASMA, and Anti-LKM-1 were negative. His pANCA was strongly positive. The MRCP was negative for PSC. His liver biopsy showed markedly active chronic hepatitis with confluent necrosis, and plasma cell predominance. His liver function normalized with initiation of prednisone and 6-MP. Case 3: A 17-year-old African-American male with a two month history of UC in remission on mesalamine presented with AST and ALT over 400, and an elevated gamma globulin fraction. His additional liver workup was negative. His ANA, ASMA, and Anti-LKM-1 were negative. His anti-actin antibodies were strongly positive. The MRCP was negative for PSC. The liver biopsy showed markedly active chronic hepatitis with interface hepatitis, confluent necrosis, and plasma cell predominance. Furthermore, there was focal bile duct paucity possibly consistent with possible incipient or small duct PSC. Prednisone and 6-MP was started concomitantly and his liver function normalized. The patient is being followed closely with serial labs and cholagiographies. Discussion: We presented three cases of AIH with preexisting well controlled UC with negative ANA, ASMA, and anti-LKM1. All three of these patients responded to prednisone, and are being maintained with 6-MP. Since up to 42% of patients with UC and AIH may develop abnormal cholangiography, close follow up with labs and imaging is required.