Primary biliary cirrhosis: clinical, pathologic, and helical CT findings in 53 patients

PURPOSE: To evaluate and compare clinical, pathologic, and helical computed tomographic (CT) findings of primary biliary cirrhosis (PBC). MATERIALS AND METHODS: The authors reviewed the medical records and CT scans of 53 patients who underwent evaluation, treatment, and orthotopic liver transplantation (OLT) at their institution. All patients underwent helical multiphase CT (total, 98 abdominal CT scans; range, one to five scans per patient). Multiple epidemiologic, clinical, and morphologic criteria were evaluated. Advanced disease was defined as hepatic insufficiency leading to OLT within the subsequent 2 years. Clinical and morphologic features were evaluated and compared in the advanced and less advanced cases of PBC. RESULTS: Common and characteristic findings included the following: 45 (85%) of the 53 patients were women with the onset of disease (diagnosis) in middle age (mean, 50.7 years; range, 26-71 years). The average time from diagnosis to OLT was 6.1 years (range, 1.5-20.0 years). CT findings in advanced PBC often resembled those seen in other forms of cirrhosis, with a small heterogeneously attenuating liver, varices, and splenomegaly. The liver in less advanced disease was usually enlarged or normal in size, with a smooth contour, little atrophy, and lacelike fibrosis and regenerative nodules in nearly one-third of the livers. Patients with less advanced disease frequently had varices (n = 33 62%]) and ascites (n = 13 24%]). Lymphadenopathy was seen in 47 (88%) patients. Hepatocellular carcinoma was found in four (8%) patients, two of whom also had chronic hepatitis C. During a follow-up period of 5-72 months (median, 46 months; mean, 42 months) after OLT, only two patients experienced recurrence of PBC. CONCLUSION: PBC is an important cause of liver failure, with distinctive clinical and CT findings that may assist diagnosis and allow adequate treatment. CT can demonstrate varices and ascites before frank cirrhosis is evident and can help evaluate the progression of the disease.