Autoimmune Hepatitis-Primary Sclerosing Cholangitis Overlap Syndrome Complicated by Crohn’s Disease

transhepatic cholangiography (PTC) with temporary biliary stenting was performed within a month of the new diagnosis for worsening cholestasis. Three months later, mycophenolate mofetil (Cellcept) was started for persistently increased transaminases. She finally began to respond with significant decrease in serum transaminase and alkaline phosphatase levels. After 3 years of successful management, she developed diarrhea and abdominal cramping. Colonoscopy revealed extensive patchy mucosal inflammation throughout the colon and terminal ileum. Mucosal biopsies demonstrated extensive crypt distortion, focal cryptitis, increased chronic inflammation, fibrino-inflammatory exudates and erosion establishing a diagnosis of Crohn’s disease (CD) ( fig. 1 ). Contrasted CT scan of the abdomen and pelvis revealed diffuse thickening of terminal ileum, cecum, ascending and transverse colonic wall and pericolic stranding consistent with active inflammatory bowel disease. It also demonstrated signs of liver cirrhosis (based on the increased caudate to right lobe ratio) as well as intraand extrahepatic biliary dilation ( fig. 2 a, b). Budesonide 9 mg daily was added to treat active CD and prednisone was discontinued. She remained symptomatic with continued diarrhea and further deterioraDear Sir Autoimmune hepatitis-primary sclerosing cholangitis (AIH-PSC) overlap syndrome is characterized by features of both conditions. Association of AIH-PSC overlap syndrome with ulcerative colitis (UC) is well recognized but is rarely seen with Crohn’s disease (CD). We report a case of a young African-American woman with AIH-PSC overlap syndrome complicated by CD that illustrates the approach to diagnosis and management of the condition. A brief discussion of the topic follows the case presentation.

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