Andrea Cox, M.D., Ph.D. and Christopher J. Hoffmann, M.D., M.P.H.
- Almost no data on epidemiology of HIV cholangiopathy in Zambia or sub-Saharan Africa
- Given association between HIV cholangiopathy and organisms prevalent in HIV+ patients in Zambia, (e.g. Cryptosporidium, microsporidia, Cyclospora, and CMV), this disease likely underdiagnosed in Zambia.
- ERCP not readily available in Zambia. Ultrasound and lab findings in appropriate clinical scenario may lead to presumptive diagnosis.
- Treatment options, besides ART, are limited in Zambia.
Zambia Information Author: Larry William Chang, MD, MPH
- Syndrome of biliary obstruction resulting from infection-associated strictures of the biliary tract. Typically occurs in pts with advanced HIV disease(CD4 <50). 4 sub-classifications:
- - Papillary strictures/stenosis (10%)
- - Sclerosing cholangitis-like (20%)
- - Papillary stenosis with sclerosing cholangitis (50-60%)
- - Extra-hepatic strictures (rare)
- Sx: RUQ pain (90%) often sharp & radiating to back (especially if strictures present). Fever (50%), low grade if present. Diarrhea common due to small bowel involvement with infectious agent.
- Signs: LFTs suggest cholestasis: mild increase in ALT/AST (2-3x ULN), total bili usually <2x ULN (jaundice rare but can be present), alk phos 5-10x ULN. LFTs normal in 20%.
- Large intrahepatic ducts most commonly involved; Cryptosporidium and CMV are usual pathogens in such cases.
- Idiopathic in ~20% of cases.
- Established with ERCP. Sensitivity of ultrasound (US) is 75-97%.
- If US positive, ERCP indicated to confirm Dx and treat. If US negative, ERCP if abd. pain is severe or w/ known CMV or Cryptosporidium infection.
- OIs (usually CMV, Cryptosporidium, or microsporidium) involved in >50% of cases.
- ERCP with sphincterotomy provides relief of Sx, but alk phos often remains high.
- Endoscopic stenting considered for pain management
- Ursodeoxycholic acid (ursodiol 300 mg PO three times a day chronically) used experimentally in HIV cholangiopathy.
- Role of ART in managing cholangiopathy is unclear, but overall survival improved by ART.
- Viral hepatitis (HAV, HBV, HCV, HDV)
CMV, HSV, EBV infection
- Heptobiliary cryptococcosis
- Mycobacterial infection of the liver (TB, MAC)
- Fatty infiltration of liver
- Drug reaction (TMP-SMX, INH, rifampin, ketoconazole, pentamidine, pyrimethamine, dapsone)
- Vanishing bile duct syndrome (associated with high bilirubin)