2290 A Unique Presentation of Severe Cholestatic Autoimmune Hepatitis With an Associated Biliary Stricture

Abstract

INTRODUCTION: Autoimmune hepatitis, usually associated with hepatocellular injury patterns, has rarely been described in current literature presenting with severe cholestasis. Even fewer cases have associated autoimmune hepatitis in patients with Mixed Connective Tissue Disease (MCTD). We describe a rare case report of a young male with suspected MCTD who presented with severe cholestatic autoimmune hepatitis with an associated biliary stricture. CASE DESCRIPTION/METHODS: A 33 yo AA male veteran with no significant PMHx presented with a 3 months of RUQ abdominal pain with associated jaundice, fatigue, and subjective fevers. On further review, he endorsed dry mouth, joint pain, and pain in his fingers in cold temperatures. He denied alcohol use, taking herbals or prescription medications. He endorsed travel to Korea. Physical examination was notable for RUQ abdominal tenderness and jaundice. Initial labs were significant for: Bilirubin: 11.6, Alk Phos: 338, AST/ALT: 1086, 116, Tylenol level: below assay, INR 1.2, platelets 213, Ferritin: 6,767, and Ceruloplasmin: 32. Acute viral hepatitis testing for A, B, C, and E were negative. HFE gene mutation was negative. Autoimmune markers included: positive ANA 1:320, positive RNP Ab, positive Smooth Muscle Ab 1:80; elevated IgG subclass 1 (2,010) and IgG subclass 2 (589). RUQ ultrasound and CT Abdomen/Pelvis showed evidence of cirrhosis. MRCP and ERCP revealed: gallbladder distension, cirrhosis of the right hepatic lobe, biliary strictures of the common hepatic and right intrahepatic bile ducts. The biliary stricture brushings were negative for malignancy. Ileocolonoscopy was unremarkable without inflammatory bowel disease. He underwent laparoscopic cholecystectomy with intraoperative liver biopsies. Interestingly, his liver biopsies revealed marked cholestasis with lymphoplasmacytic portal inflammation and minimal fibrosis (stage 1-2) despite imaging suggestive of cirrhosis. Ultimately , these findings were consistent with severe cholestatic autoimmune hepatitis with an associated biliary stricture in a patient with suspected underlying MCTD. DISCUSSION: Our patients demographics, travel and pattern of liver injury posed a diagnostic dilemma for diagnosing autoimmune hepatitis. After a thorough evaluation for the etiology, his liver histopathology, positive autoimmune markers, and favorable response to steroids were all compatible with the diagnosis of cholestatic autoimmune hepatitis with a biliary stricture in a patient with suspected MCTD.