Acquired immunodeficiency syndrome cholangiopathy: spectrum of disease

Reports of biliary tract abnormalities in patients with acquired immunodeficiency syndrome (AIDS) are infrequent. Nine patients with features of papillary stenosis and sclerosing cholangitis and one patient with primary bile duct lymphoma were previously described. Since those reports, an additional 16 AIDS patients with clinical and laboratory features suggestive of biliary tract disease have been evaluated. The findings in these 26 patients form the basis of this study.

The patients, 25 men and one woman, with right upper quadrant abdominal pain, fevers, or elevated serum alkaline phosphatase levels, or any combination of the three symptoms, underwent endoscopic retrograde cholangiopancreatography and ampullary biopsy.

Twenty of these 26 (77%) patients were found to have markedly abnormal cholangiograms. Four patterns of cholangiographic abnormalities were identified: sclerosing cholangitis and papillary stenosis (10 patients), papillary stenosis alone (three patients), sclerosing cholangitis alone (four patients), and long extrahepatic bile duct strictures (three patients). An AIDS-associated pathogen or malignancy was identified in 11 patients (55%), with cytomegalovirus being the most common (five patients). Demographic, clinical, and laboratory features were not significantly different between AIDS patients with cholangiopathy and those without cholangiopathy. However, 15 of 20 patients with cholangiographic abnormalities (versus only one of six patients without such abnormalities) had ductal dilation on ultrasound or computed tomography, or both.

Clinicians should be aware that profound biliary tract abnormalities may develop in a large number of human immunodeficiency virus-infected patients. Confirmation of the anatomic abnormalities, determination of correct etiology, and effective therapy may depend upon early endoscopic cholangiography.