ReviewDiagnostic criteria of autoimmune hepatitis
Introduction
Autoimmune hepatitis (AIH), a progressive inflammatory hepatopathy and important cause of end-stage liver disease, was first described in a group of young women in 1950 by the Swedish professor Jan Waldenström. The patients were afflicted by a severe form of un-resolving hepatitis characterised by markedly elevated serum immunoglobulin levels [1]. The observation that a proportion of patients in similar cohorts were positive for lupus erythematosus cells and anti-nuclear autoantibodies (ANA), led to the hypothesis that a loss of immune tolerance was at the basis of this condition [2].
The most typical features of AIH are female preponderance, hypergammaglobulinaemia, seropositivity for circulating autoantibodies and a picture of interface hepatitis on histology [3]. AIH responds to immunosuppressive treatment in the majority of cases. Treatment should be instituted promptly upon diagnosis. If left untreated, AIH usually progresses to liver failure requiring transplantation. Two types of AIH are distinguished according to serological profile: type 1 AIH (AIH-1) patients are positive for ANA and/or anti-smooth muscle antibody (SMA), and type 2 AIH (AIH-2) patients are defined by the positivity for anti-liver kidney microsomal type 1 antibody (anti-LKM-1) and/or for anti-liver cytosol type 1 antibody (anti-LC-1) [4].
Section snippets
Epidemiology
AIH occurs globally, affecting children and adults of all ages [5], [6] and both sexes, although it is more commonly found in females [7].
Estimates of the prevalence of AIH were mired by a lack of standardization before the introduction of the International Autoimmune Hepatitis Group (IAIHG) Scoring System in the early 1990s [8], [9]. Moreover, early studies did not exclude patients with hepatitis C virus infection. The first study to utilize the IAIHG Scoring System reported a prevalence of
Clinical presentation and natural history
The mode of presentation of AIH is diverse and highly variable [12], although the majority of adult patients manifest with an insidious onset characterised by progressive fatigue, relapsing jaundice, amenorrhea, weight loss and occasionally arthralgia [13]. Complications of portal hypertension, such as gastrointestinal bleeding or hypersplenism can sometimes be apparent [14]. Approximately 25% of patients are completely asymptomatic and are diagnosed after incidental discovery of abnormal liver
Scoring systems
The diagnosis of AIH is based on the presence of elevated serum transaminase and IgG levels, autoantibody seropositivity and histological evidence of interface hepatitis.
This tenet has been factored into the IAIHG diagnostic criteria for AIH [8], [9], which were originally developed for use in the research setting. This scoring system incorporates a number of positive and negative scores, enabling the researcher to grade clinical, laboratory and histological features of AIH. The score has also
Treatment
With the exception of fulminant presentation with encephalopathy, AIH responds to immunosuppressive treatment whatever the degree of liver impairment [20].
Standard treatment, consisting of prednisolone alone or in combination with azathioprine, induces remission (i.e. normal transaminases and IgG levels) in around 80% of the patients, including those with cirrhosis [20], [32]. Once achieved, remission can be maintained with azathioprine alone [20].
In treatment failure, other drugs have been
References (40)
- et al.
Liver autoimmune serology: a consensus statement from the committee for autoimmune serology of the International Autoimmune Hepatitis Group
J Hepatol
(2004) - et al.
Autoimmune hepatitis (AIH) in the elderly: a systematic retrospective analysis of a large group of consecutive patients with definite AIH followed at a tertiary referral centre
J Hepatol
(2006) - et al.
Autoimmune hepatitis: a comprehensive review
J Autoimmun
(2013) - et al.
International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis
J Hepatol
(1999) - et al.
Prevalence of autoimmune liver disease in Alaska Natives
Am J Gastroenterol
(2002) - et al.
Fulminant hepatic failure as the initial presentation of acute autoimmune hepatitis
Clin Gastroenterol Hepatol
(2004) - et al.
Severe autoimmune hepatitis first presenting in the early post partum period
Clin Gastroenterol Hepatol
(2004) - et al.
Autoantibodies against glucuronosyltransferases differ between viral hepatitis and autoimmune hepatitis
Gastroenterology
(1996) - et al.
Antibodies to conformational epitopes of soluble liver antigen define a severe form of autoimmune liver disease
Hepatology
(2002) - et al.
Identification of target antigen for SLA/LP autoantibodies in autoimmune hepatitis
Lancet
(2000)
Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study
Hepatology
Autoimmune hepatitis after liver transplantation
Clin Gastroenterol Hepatol
T-regs in autoimmune hepatitis-systemic lupus erythematosus/mixed connective tissue disease overlap syndrome are functionally defective and display a Th1 cytokine profile
J Autoimmun
The interplay between regulatory and effector T cells in autoimmune hepatitis: implications for innovative treatment strategies
J Autoimmun
Autoimmune hepatitis: contrasts and comparisons in children and adults — a comprehensive review
J Autoimmun
Clinical significance of autoantibodies in autoimmune hepatitis
J Autoimmun
Liver auto-immunology: the paradox of autoimmunity in a tolerogenic organ
J Autoimmun
Leber, Blutprotein und Nahrungseiweiss
Dtsch Ges Z Verdan Stoffwechselkr
Toward diagnostic criteria for autoimmune hepatitis
Hepatology
Autoimmune hepatitis
N Engl J Med
Cited by (91)
Diagnosis of Autoimmune Hepatitis
2024, Clinics in Liver DiseaseConventional liver imaging in hepatitis
2023, Comprehensive Guide to Hepatitis AdvancesAutoimmune Hepatitis
2023, MacSween's Pathology of the Liver, Eighth EditionRational development and application of biomarkers in the field of autoimmunity: A conceptual framework guiding clinicians and researchers
2022, Journal of Translational AutoimmunityAntinuclear antibodies (ANA) as a criterion for classification and diagnosis of systemic autoimmune diseases
2022, Journal of Translational AutoimmunityRegulatory T cells in autoimmune hepatitis: Un updated overview
2021, Journal of Autoimmunity