Open AccessConcurrent primary biliary cirrhosis and autoimmune hepatitis presenting as subfulminant hepatic failure
Author(s) -
Jason K. Hou,
M. Eric Gershwin,
Linda K. Green,
Boris Yoffe
Publication year - 2009
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr.08.2008.0767
Subject(s) - medicine , overlap syndrome , autoimmune hepatitis , primary biliary cirrhosis , ursodeoxycholic acid , prednisone , gastroenterology , asymptomatic , cirrhosis , hepatitis , disease
Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) overlap syndrome may be found in a significant percentage of patients with either PBC or AIH. However, it is conceivable that most cases of PBC-AIH overlap syndrome are a concurrent manifestation of both diseases which therefore requires treatment of both disease entities. While most cases are found in asymptomatic patients or in patients who have been previously diagnosed with PBC, our patient presented with concurrent PBC and AIH resulting in subfulminant liver failure that responded to treatment with prednisone and ursodeoxycholic acid (UDCA). Extended 4-year follow-up and treatment with UDCA confirmed the diagnosis of PBC and demonstrated serological resolution of AIH.