title: Vanishing bile duct syndrome in human immunodeficiency virus infected adults: a report of two cases.
abstract: Vanishing bile duct syndrome (VBDS) is a group of rare disorders characterized by ductopenia, the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis. Described in association with medications, autoimmune disorders, cancer, transplantation, and infections, the specific mechanisms of disease are not known. To date, only 4 cases of VBDS have been reported in human immunodeficiency virus (HIV) infected patients. We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases. Presentation includes hyperbilirubinemia, normal liver imaging, and negative viral and autoimmune hepatitis studies. In HIV-infected subjects, VBDS occurred at a range of CD4+ T-cell counts, in some cases following initiation or change in antiretroviral therapy. Lymphoma was associated with two cases; nevirapine, antibiotics, and viral co-infection were suggested as etiologies in the other cases. In HIV-positive patients with progressive cholestasis, early identification of VBDS and referral for transplantation may improve outcomes.
authors: Section of Infectious Diseases, Department of Medicine, Wake Forest School of Medicine, Winston-Salem, NC 27109-7205, USA.
authors: Ana Paula
authors: AP
authors: Oppenheimer
authors: Christopher
authors: C
authors: Koh
authors: Mary
authors: M
authors: McLaughlin
authors: John C
authors: JC
authors: Williamson
authors: Thomas D
authors: TD
authors: Norton
authors: Jennifer
authors: J
authors: Laudadio
authors: Theo
authors: T
authors: Heller
authors: David E
authors: DE
authors: Kleiner
authors: Kevin P
authors: KP
authors: High
authors: Caryn G
authors: CG
authors: Morse
mesh_terms: D000328:Adult; D044966:Anti-Retroviral Agents / Q000627:therapeutic use; D001649:Bile Duct Diseases / Q000150:complications* / Q000175:diagnosis*; D001706:Biopsy; D015496:CD4-Positive T-Lymphocytes / Q000166:cytology; D002779:Cholestasis; D005260:Female; D015658:HIV Infections / Q000150:complications*; D006801:Humans; D007565:Jaundice / Q000150:complications; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000209:etiology; D008297:Male; D011379:Prognosis; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D044966:Anti-Retroviral Agents
keywords: Antiretroviral therapy; Ductopenia; Human immunodeficiency virus; Liver biopsy; Vanishing bile duct syndrome