Case Report
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World J Gastroenterol. Jan 7, 2013; 19(1): 115-121
Published online Jan 7, 2013. doi: 10.3748/wjg.v19.i1.115
Vanishing bile duct syndrome in human immunodeficiency virus infected adults: A report of two cases
Ana Paula Oppenheimer, Christopher Koh, Mary McLaughlin, John C Williamson, Thomas D Norton, Jennifer Laudadio, Theo Heller, David E Kleiner, Kevin P High, Caryn G Morse
Ana Paula Oppenheimer, John C Williamson, Kevin P High, Section of Infectious Diseases, Department of Medicine, Wake Forest School of Medicine, Winston-Salem, NC 27109-7205, United States
Christopher Koh, Theo Heller, Liver Diseases Branch, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health Clinical Center, Bethesda, MD 20892-1403, United States
Mary McLaughlin, Laboratory of Immunoregulation, National Institute of Allergy and Infectious Diseases, National Institutes of Health Clinical Center, Bethesda, MD 20892-1403, United States
Thomas D Norton, Division of Infectious Diseases and Immunology, Department of Medicine, New York University School of Medicine, New York, NY 10016, United States
Jennifer Laudadio, Laboratory of Pathology, Wake Forest School of Medicine, Winston-Salem, NC 27109-7205, United States
David E Kleiner, Laboratory of Pathology, National Cancer Institute, National Institutes of Health Clinical Center, Bethesda, MD 20892-1403, United States
Caryn G Morse, Department of Critical Care Medicine, National Institutes of Health Clinical Center, Bethesda, MD 20892-1403, United States
Author contributions: Oppenheimer AP, Koh C, McLaughlin M, Williamson JC, Norton TD, Heller T, High KP and Morse CG provided clinical support for the patients; Laudadio J and Kleiner DE performed pathological examinations; Oppenheimer AP, Williamson JC, and Morse CG analyzed the data; Oppenheimer AP, Koh C Morse CG wrote the paper; Williamson JC and High KP provided critical revision.
Supported by The Intramural Research Programs of the National Institutes of Health Clinical Center and the National Institute of Allergy and Infectious Diseases
Correspondence to: Caryn G Morse, MD, MPH, Department of Critical Care Medicine, National Institutes of Health Clinical Center, 9000 Rockville Pike, Building 10, 5A06, MSC 1403, Bethesda, MD 20892-1403, United States. cmorse@mail.nih.gov
Telephone: +1-301-4966028 Fax: +1-301-4021137
Received: March 13, 2012
Revised: July 2, 2012
Accepted: August 3, 2012
Published online: January 7, 2013
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.

Keywords: Human immunodeficiency virus, Antiretroviral therapy, Vanishing bile duct syndrome, Ductopenia, Liver biopsy