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Published 17 March 2009
Cite this as: BMJ Case Reports 2009 [doi:10.1136/bcr.08.2008.0767]
Copyright © 2009 by the BMJ Publishing Group Ltd.

Unusual presentation of more common disease/injury

Concurrent primary biliary cirrhosis and autoimmune hepatitis presenting as subfulminant hepatic failure

Jason Ken Hou1, M Eric Gershwin2, Linda K Green3, Boris Yoffe4

1 Baylor College of Medicine, Gastroenterology and Hepatology, 8.35, 1709 Dryden Road, Houston, TX 77030, USA
2 University of California at Davis, Division of Rheumatology, Allergy and Clinical Immunology, 6510, 451 Health Sciences Drive, Davis, CA 95616, USA
3 Baylor College of Medicine, Pathology, VA Medical Center, Houston, TX 77030, USA
4 Baylor College of Medicine, Gastroenterology and Hepatology, 151B, VA Medical Center, 2002 Holcombe Blvd Building, Houston, TX 77030, USA

Correspondence to:
Jason Ken Hou, jkhou{at}bcm.tmc.edu

SUMMARY

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.


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