(See also Overview of Biliary Function Overview of Biliary Function The liver produces about 500 to 600 mL of bile each day. Bile is isosmotic with plasma and consists primarily of water and electrolytes but also organic compounds: bile salts, phospholipids... read more .)
Before the advent of antiretroviral therapy, cholangiopathy occurred in 25% of patients with AIDS AIDS Human immunodeficiency virus (HIV) infection results from 1 of 2 similar retroviruses (HIV-1 and HIV-2) that destroy CD4+ lymphocytes and impair cell-mediated immunity, increasing risk of certain... read more , especially in those with a low CD4 count (< 100/mcL). The most common pathogen is Cryptosporidium parvum. Others include cytomegalovirus Cytomegalovirus (CMV) Infection Cytomegalovirus (CMV, human herpesvirus type 5) can cause infections that have a wide range of severity. A syndrome of infectious mononucleosis that lacks severe pharyngitis is common. Severe... read more , microsporidia Microsporidiosis Microsporidiosis is infection with microsporidia. Symptomatic disease develops predominantly in patients with AIDS and includes chronic diarrhea, disseminated infection, and corneal disease... read more , and Cyclospora species. Papillary stenosis or intrahepatic or extrahepatic sclerosing cholangitis Sclerosing Cholangitis Sclerosing cholangitis refers to chronic cholestatic syndromes characterized by patchy inflammation, fibrosis, and strictures of the intrahepatic and extrahepatic bile ducts. Progression obliterates... read more develops in most patients. Over half have both.
Common symptoms include right upper quadrant and epigastric pain and diarrhea. A few patients have fever and jaundice Jaundice Jaundice is a yellowish discoloration of the skin and mucous membranes caused by hyperbilirubinemia. Jaundice becomes visible when the bilirubin level is about 2 to 3 mg/dL (34 to 51 micromol/L)... read more . Severe pain usually indicates papillary stenosis. Milder pain suggests sclerosing cholangitis Sclerosing Cholangitis Sclerosing cholangitis refers to chronic cholestatic syndromes characterized by patchy inflammation, fibrosis, and strictures of the intrahepatic and extrahepatic bile ducts. Progression obliterates... read more . The diarrhea reflects small-bowel infection, often cryptosporidiosis Cryptosporidiosis Cryptosporidiosis is infection with the protozoan Cryptosporidium. The primary symptom is watery diarrhea, often with other signs of gastrointestinal distress. Illness is typically self-limited... read more .
Imaging Imaging Tests of the Liver and Gallbladder Imaging is essential for accurately diagnosing biliary tract disorders and is important for detecting focal liver lesions (eg, abscess, tumor). It is limited in detecting and diagnosing diffuse... read more usually begins with ultrasonography, which is noninvasive and very accurate (> 95%). However, ERCP is usually necessary. ERCP provides the diagnosis, enables clinicians to take a sample for small-bowel biopsy for identification of the causative organism, and provides a therapeutic opportunity to relieve strictures. CT and magnetic resonance cholangiopancreatography likely have supportive roles.
Abnormal liver test Laboratory Tests of the Liver and Gallbladder Laboratory tests are generally effective for the following: Detecting hepatic dysfunction Assessing the severity of liver injury Monitoring the course of liver diseases and the response to treatment... read more results (especially a high alkaline phosphatase level) are consistent with cholestasis.
Endoscopic sphincterotomy, often done during endoscopic retrograde cholangiopancreatography, can markedly relieve pain, jaundice Jaundice Jaundice is a yellowish discoloration of the skin and mucous membranes caused by hyperbilirubinemia. Jaundice becomes visible when the bilirubin level is about 2 to 3 mg/dL (34 to 51 micromol/L)... read more , and cholangitis Choledocholithiasis and Cholangitis Choledocholithiasis is the presence of stones in bile ducts; the stones can form in the gallbladder or in the ducts themselves. These stones cause biliary colic, biliary obstruction, gallstone... read more in patients with papillary stenosis. Isolated or dominant strictures can be stented endoscopically. Antimicrobial therapy is given to treat the infection but alone does not reduce the biliary tract damage or relieve symptoms.
Ursodeoxycholic acid may have a role in treating intrahepatic ductal sclerosis and cholestasis.