Variant forms of cholestatic diseases involving small bile ducts in adults

OBJECTIVE: Cholestasis may result from diverse etiologies. We review chroni c cholestatic disorders involving small intrahepatic bile ducts in the adul t ambulatory care setting. Specifically, we discuss variant forms of primar y biliary cirrhosis (PBC); and primary sclerosing cholangitis (PSC) as well as other conditions that may present diagnostic and therapeutic difficulti es. METHODS: We conducted a MEDLINE search of the literature (1981-1997) and re viewed the experiences at the Mayo Clinic. All articles were selected that discussed anti-mitochondrial antibody (AMA)-negative PBC, small-duct PSC (f ormerly pericholangitis), and idiopathic adulthood ductopenia. RESULTS: The most common chronic cholestatic liver diseases affecting adult s are PBC and PSC. Patients without the hallmarks of either syndrome are di agnosed according to their clinical and histological characteristics. Autoi mmune cholangitis is diagnosed if clinical and histological features are co mpatible with PBC but autoantibodies other than AMA are present. Isolated s mall duct PSC is diagnosed if patients have inflammatory bowel disease, bio psy features compatible with PSC, but a normal cholangiogram. If ductopenia (absence of interlobular bile ducts in small portal tracts) is found histo logically in the absence of PSC, inflammatory bowel disease, and other spec ific cholestatic syndromes such as drug reaction or sarcoidosis, the most l ikely diagnosis is idiopathic adulthood ductopenia. CONCLUSIONS: Based on these definitions, an algorithm for diagnosis and the rapy in patients with laboratory evidence of chronic cholestasis may be con structed, pending results of further investigations into the etiopathogenes is of these syndromes. (C) 2000 by Am. Coll. of Gastroenterology.