Primary Sclerosing Cholangitis -- Approach to Diagnosis

Ian L. Steele, MD; Cynthia Levy, MD; Keith D. Lindor, MD


April 25, 2007

Case Descriptions

Case 1

A 19-year-old white man was found to have abnormal serum liver biochemistry test results 3 years ago when he was first diagnosed as having ulcerative colitis. He denied any previous transfusions, gallstone disease, intravenous drug use, tattoos, or significant alcohol consumption. The patient had no family history of liver disease and, with the exception of his ulcerative colitis, he reported that he was healthy. Even after stopping all medications, his liver function test results remained abnormal. His laboratory studies were significant for increased serum alkaline phosphatase (ALP) and aminotransferase levels elevated to 3 times the upper limit of normal. His bilirubin level was normal (< 1 mg/dL). Endoscopic retrograde cholangiopancreatography (ERCP) was performed and showed multiple stenoses and dilatations throughout the intrahepatic biliary system, with a pruning type pattern. The common bile duct and extrahepatic portion of the common hepatic duct appeared normal, without strictures. These biliary findings coupled with the presence of inflammatory bowel disease (IBD) and the absence of any alternative etiology led to a confident diagnosis of PSC.

Case 2

A 47-year-old white woman was found to have abnormal liver function test results during a routine health maintenance examination. She had always been asymptomatic and denied any social risk factors for acquired liver disease. Laboratory studies showed the following: serum aspartate aminotransferase (AST), 68 U/mL (normal, 10-39 U/mL); serum alanine aminotransferase (ALT), 50 U/mL (normal, 10-39 U/mL); serum total bilirubin, 0.8 mg/dL (normal, 0.1-1.0 mg/dL); and ALP, 289 U/L (normal, 45-129 U/L). Serologic markers, including antimitochondrial antibody and antinuclear antibody, were normal, as were immunoglobulins, including IgG4 and carbohydrate antigen 19-9 (CA19-9). Magnetic resonance cholangiopancreatography (MRCP) was performed and showed hepatic ductal dilatation, with no evidence of mass lesions. The patient subsequently underwent ERCP at an outside facility, which showed right hepatic ductal dilatation without visualization of the left hepatic ducts. This finding was indicative for obstruction, and malignancy could not be ruled out. Therefore, the patient was referred to our institution for tumor resection. A repeat MRCP was performed and showed intrahepatic ductal dilatation with tortuous and diffuse dilatation most consistent with PSC. Focal obstruction at the confluence of right and left hepatic ducts was still indicative for a Klatskin tumor. However, repeat ERCP showed tight strictures of the left hepatic duct and the first division of the right hepatic duct. Several smaller strictures were noted in the smaller intrahepatic ducts. Biopsies and cytology specimens obtained during this ERCP showed no evidence of malignancy. This patient has been observed for 1 year and her benign course corroborated a diagnosis of PSC.


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