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Semin Liver Dis 2007; 27(2): 214-226
DOI: 10.1055/s-2007-979472
Copyright © 2007 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Guidelines for Therapy of Autoimmune Liver Disease

Hiromi Ishibashi1 , Atsumasa Komori1 , Shinji Shimoda2 , M. Eric Gershwin3
  • 1Clinical Research Center, National Hospital Organization (NHO), Nagasaki Medical Center, and Department of Hepatology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
  • 2Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, Fukuoka, Japan
  • 3Division of Rheumatology, Allergy, and Clinical Immunology, University of California at Davis School of Medicine, Genome and Biomedical Sciences Facility, Davis, California
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Publication History

Publication Date:
22 May 2007 (online)

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ABSTRACT

The principle of therapy for chronic inflammatory liver diseases is the removal of causal agents. For autoimmune liver diseases, however, total removal of causal agents and immune cells is impossible. Therefore, autoimmune liver diseases are presently treated by suppression of the immune response. Autoimmune hepatitis is characteristically responsive to corticosteroids, often used in combination with azathioprine to obtain a steroid-sparing effect. For primary biliary cirrhosis, ursodeoxycholic acid is safe and is the first choice for treatment. Treatment of this autoimmune liver disease should also address various symptoms and complications arising from any associated autoimmune diseases, particularly cholestasis and cirrhosis-related complications. For primary sclerosing cholangitis there are no established immunomodulatory therapies, but medical, endoscopic, and surgical treatments are applicable to this disease. Liver transplantation becomes indicated during the eventual end stages of each of these immune-mediated liver diseases.

KEYWORDS

Treatment - guideline - autoimmune hepatitis - primary biliary cirrhosis - primary sclerosing cholangitis - overlapping syndrome

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1 Comments on the AASLD guideline[4]: for precise explanation refer to the original article (ref. 4).

1 I, evidence from multiple well-designed randomized controlled trials, each involving a number of participants to be of sufficient statistical power.

1 II, evidence from at least one large, well-designed clinical trial with or without randomization, from cohort or case-control analytic studies, or from well-designed meta-analysis.

1 III, evidence based on clinical experience, descriptive studies, or reports of expert committees.

1 IV, not rated.

2 Comments on the AASLD guideline[51]:

2 Categories reflecting the evidence to support the use of a guideline recommendation (category and definition):

2 A, Survival benefit; B, improved diagnosis; C, improvement in quality of life; D, relevant pathophysiologic parameters improved; E, impacts, cost of health care.

2 These standardized guidelines of the practice guideline of the AASLD have been modified from the categories of the Infectious Diseases Society of America's quality standards (cited from ref. 50). For precise explanation, refer to the original article (ref. 51).

Hiromi IshibashiM.D. 

Director General, Clinical Research Center, National Hospital Organization (NHO), Nagasaki Medical Center

Kubara 2-1001-1, Omura, Nagasaki, 856-8562, Japan

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