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정승규 ( Seung Kyu Chung ),장재영 ( Jae Young Jang ),정승원 ( Soung Won Jeong ),심찬섭 ( Chan Sup Shim ),김부성 ( Boo Sung Kim ),진윤미 ( Yoon Mi Jeen ),진소영 ( So Young Jin ) 대한내과학회 2009 대한내과학회지 Vol.77 No.6
저자들은 전신쇠약감 등 비특이적인 증상과 간기능 이상이 있는 환자를 대상으로 임상양상, 혈청학적, 면역학적 조직학적인 검사를 통해 AMA 음성인 자가면역성 담관염으로 진단된 네 개의 증례를 경험하여 보고하는 바이다. Autoimmune cholangitis is a disease with clinicopathologic features of primary biliary cirrhosis without antimitochondrial antibodies and, frequently, with high antinuclear antibody titers. Alternative terms have been suggested, including antimitochondrial antibody-negative primary biliary cirrhosis, immunocholangitis, autoimmune cholangiopathy, and autoimmune hepatobiliary overlap syndrome type 1. The question of whether autoimmune cholangitis is distinct from primary biliary cirrhosis and autoimmune hepatitis or is an antimitochondrial antibody-negative variant of primary biliary cirrhosis and autoimmune hepatitis is controversial. This case report describes the clinical, immunohistochemical, and pathological findings in four patients with autoimmune cholangitis and reviews the literature. (Korean J Med 77:750-754, 2009)