![]() ![]() The prognosis is poor with progression to biliary cirrhosis in the majority of cases. Immune mediated mechanisms have been proposed. VBDS has been described in association with autoimmune disorders, medications, cancer, transplantation, and infection, though idiopathic cases have also been described. Vanishing bile duct syndrome (VBDS) is a term that refers to a group of rare disorders characterized by progressive destruction and disappearance of the intrahepatic bile ducts leading to intrahepatic cholestasis. ![]() In HIV-positive patients with progressive cholestasis, early identification of VBDS and referral for transplantation may improve outcomes. Lymphoma was associated with two cases nevirapine, antibiotics, and viral co-infection were suggested as etiologies in the other cases. In HIV-infected subjects, VBDS occurred at a range of CD4+ T-cell counts, in some cases following initiation or change in antiretroviral therapy. Presentation includes hyperbilirubinemia, normal liver imaging, and negative viral and autoimmune hepatitis studies. We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases. To date, only 4 cases of VBDS have been reported in human immunodeficiency virus (HIV) infected patients. ![]() Described in association with medications, autoimmune disorders, cancer, transplantation, and infections, the specific mechanisms of disease are not known. 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. ![]()
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