Vanishing bile duct syndromes in adults Marek Hartleb Medical Science Review - Hepatologia 2005; 5 57-64 aaICID: 16113
IC™ Value: 1.86
Abstract provided by Publisher
Katedra i Klinika Gastroenterologii, Śląska Akademia Medyczna, Katowice
Epithelium of small biliary ducts is an easy target to autoimmune, toxic or ischemic injuries as well as to viral, bacterial and parasitic agents. Predominance of cholangiocytic apoptosis and bile duct destruction over regenerative properties underlies vanishing bile duct syndromes (VBDS). These syndromes lead to histological ductopenia, which is defined by an absence of interlobular bile ducts in more than 50% of portal triads. Clinically and histologically ductopenia generates heterogeneous syndromes ranging from self-limited to rapidly progressive forms. In the latter VBDS results in porto-portal fibrosis, cirrhosis and hepatic failure. In majority of cases ductopenia may be attributed to autoimmune diseases such like primary biliary cirrhosis, primary sclerosing cholangitis, autoimmune cholangitis, chronic rejection of liver allograft or graft-versus-host disease. Idiopathic adulthood ductopenia is recognized in small minority of patients with VBDS, in whom the specific diagnosis cannot be established. This article reviews autoimmune, infective, granulomatous, drug-induced, toxic, ischemic, neoplastic and genetic causes of destructive cholangiopathies occurring in adults and associated with ductopenic reactions.