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