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AIDS cholangiopathy

Nezam H Afdhal, MD, FRCPI
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Shilpa Grover, MD, MPH, AGAF


AIDS cholangiopathy is a syndrome of biliary obstruction resulting from infection-related strictures of the biliary tract [1-3]. AIDS cholangiopathy occurred in as many as 26 percent of AIDS patients prior to the advent of highly active antiretroviral therapy (HAART) [4,5]; the current incidence of cholangiopathy is not known, but has decreased in the era of potent antiretroviral therapy (ART) [5].


The organism most closely associated with AIDS cholangiopathy is Cryptosporidium parvum; other pathogens that have been identified include Microsporidium, cytomegalovirus (CMV), and Cyclospora cayetanensis [2,6-10]. Involvement of the large intrahepatic ducts is usually associated with C. parvum and CMV infection [11].


AIDS cholangiopathy is usually seen in patients with a CD4 count well below 100/mm3 and may be their presenting manifestation [8]. Affected patients typically present with right upper quadrant and epigastric pain and diarrhea; fever and jaundice are less common, occurring in 10 to 20 percent of patients. The severity of the abdominal pain varies with the biliary tract lesion. Severe abdominal pain is indicative of papillary stenosis [12], while milder abdominal pain is usually associated with intrahepatic and extrahepatic sclerosing cholangitis without papillary stenosis (image 1).

The diarrhea in AIDS cholangiopathy is due to small bowel involvement with the infectious agent and may be the initial presenting feature. In one study of 95 patients with diarrhea due to Cryptosporidium, 20 (23 percent) subsequently developed biliary tract disease [13]. (See "Epidemiology, clinical manifestations, and diagnosis of cryptosporidiosis".)

Laboratory studies — Liver function tests in AIDS cholangiopathy are usually indicative of cholestasis. (See "Diagnostic approach to the adult with jaundice or asymptomatic hyperbilirubinemia".) In one series, for example, the following findings were noted [2]:

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Literature review current through: Nov 2017. | This topic last updated: Jan 10, 2017.
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