Autoimmune hepatitis and its variant syndromes
Open Access
- 1 October 2001
- Vol. 49 (4) , 589-594
- https://doi.org/10.1136/gut.49.4.589
Abstract
Three types of AIH have been proposed based on immunoserological findings but only two types have mutually exclusive autoantibodies and different clinical profiles.9 Type 1 AIH is the most common form of the disease worldwide and is associated with antinuclear antibodies (ANA) and/or smooth muscle antibodies (SMA). It affects all age groups and is associated with human leucocyte antigen (HLA) DR3 (DRB1*0301) and DR4 (DRB1*0401) in Caucasoid Northern European and North American patients.10 11 DRB1*0301 andDRB1*0401 influence disease expression and behaviour as well as susceptibility. Caucasoid patients with type 1 AIH and DRB1*0301 are younger, and have a higher frequency of treatment failure,12 relapse after drug withdrawal,13 and requirement for liver transplantation than patients with other alleles.14 In contrast, patients with DRB1*0401 are typically older, frequently have concurrent autoimmune diseases, and respond better to corticosteroids than counterparts withDRB1*0301.15Keywords
This publication has 141 references indexed in Scilit:
- Clonal accumulation of Vβ5.1-positive cells in the liver of a patient with autoimmune cholangiopathyLiver International, 2008
- Members of the glutathione S-transferase gene family are antigens in autoimmune hepatitisGastroenterology, 1998
- Ursodiol for Primary Sclerosing CholangitisNew England Journal of Medicine, 1997
- Simultaneous occurrence of primary sclerosing cholangitis and autoimmune chronic active hepatitis in a patient with ulcerative colitisDigestive Diseases and Sciences, 1992
- Autoantibodies and response to α-interferon in patients with chronic viral hepatitisJournal of Hepatology, 1990
- Association of Primary Sclerosing Cholangitis with HLA-DRw52aNew England Journal of Medicine, 1990
- Clinical Variation of Autoimmune Polyendocrinopathy–Candidiasis–Ectodermal Dystrophy (APECED) in a Series of 68 PatientsNew England Journal of Medicine, 1990
- Frequency and significance of antimitochondrial antibodies in severe chronic active hepatitisDigestive Diseases and Sciences, 1986
- Liver disease associated with anti-liver-kidney microsome antibody in childrenThe Journal of Pediatrics, 1986
- Manifestations of nonsuppurative cholangitis in chronic hepatobiliary diseases: morphologic spectrum, clinical correlations and terminologyLiver International, 1984