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Copyright ©2009 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. May 21, 2009; 15(19): 2314-2328
Published online May 21, 2009. doi: 10.3748/wjg.15.2314
Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment
Albert J Czaja, Yusuf Bayraktar
Albert J Czaja, Yusuf Bayraktar, Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota, United States and the Department of Gastroenterology, Hacettepe University School of Medicine, Ankara 06100, Turkey
Author contributions: Bayraktar Y conceived the project, suggested the content of the review, co-wrote the manuscript, provided a photomicrograph, and described the Turkish perspective; Czaja AJ wrote the draft, designed the tables, provided photomicrographs, and co-wrote the final version.
Correspondence to: Yusuf Bayraktar, MD, Professor and Head, Department of Gastroenterology, Hacettepe University School of Ankara, Ankara 06100, Turkey. bayrak@hacettepe.edu.tr
Telephone: +90-532-4323966
Fax: +90-312-4429829
Received: February 23, 2009
Revised: April 7, 2009
Accepted: April 14, 2009
Published online: May 21, 2009
Abstract

Non-classical manifestations of autoimmune hepatitis can delay diagnosis and treatment. Our aims were to describe the clinical phenotypes that can confound the diagnosis, detail scoring systems that can ensure their recognition, and outline advances in treatment that can improve their outcome. Prime source and review articles in English were selected through Medline from 1970-2008 and assimilated into personal libraries spanning 32 years. Acute severe or asymptomatic presentations and atypical histological findings, including centrilobular zone 3 necrosis and concurrent bile duct changes, are compatible with the diagnosis. Cholangiographic abnormalities may be present in children and adults with the disease, and autoimmune hepatitis must be considered in patients without autoantibodies or with antimitochondrial antibodies and no other cholestatic features. Asymptomatic patients frequently become symptomatic; mild disease can progress; and there are no confident indices that justify withholding treatment. Two diagnostic scoring systems with complementary virtues have been developed to evaluate patients with confusing features. Normal liver tests and tissue constitute the optimal end point of treatment, and the first relapse is an indication for long-term azathioprine therapy. Cyclosporine, tacrolimus and mycophenolate mofetil are promising salvage therapies, and budesonide with azathioprine may be a superior frontline treatment. We conclude that the non-classical phenotypes of autoimmune hepatitis can be recognized promptly, diagnosed accurately, and treated effectively.

Keywords: Non-classical phenotypes; Scoring systems; Treatment strategies