HLA A1, B8, DR3 extended haplotypes in autoimmune chronic hepatitis

Tait, Brian D.; Mackay, Ian R.; Board, Philip G.; Coggan, Marjorie; Emery, P; Eckardt, Glenn S.

doi:10.1016/0016-5085(89)90088-7

Cited by 32 publications

(9 citation statements)

References 9 publications

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“…The classical extended HLA haplotype HLA A1, B8 and DR3 and the HLA-DR4 allotype have been recognised as an important risk factor for the development of AIH [28,29] . Many of the immunological diseases that occur with AIH, namely autoimmune thyroiditis, RA, Sjogren's syndrome and IBD have been associated with this extended haplotype [30][31][32][33][34][35][36] .…”

Section: Effects Of Aih Types Age Gender and Genetic Backgroundmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant ‘autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH. For a majority of patients, these joint symptoms resolve within days of the introduction of immunosuppressive therapy. Rarer features at index presentation include a maculopapular skin rash and unexplained fever, which are features that tend to resolve quickly with treatment. Interestingly, joint pain and stiffness are also well recognised in the context of steroid withdrawal and cessation in AIH. The occasional co-presentation of AIH with coeliac disease is clinically important (1-6%), since for some patients, there is a risk of immunosuppression malabsorption, thus delaying effective treatment. Similarly, the co-existence of selective IgA deficiency (IgAD) can occur in patients with coeliac disease or in isolation. Selective IgAD as a co-existing extraheaptic feature seems to be more common in paediatric patients with AIH. For these patients, they are at an increased risk of respiratory and sinus infections. Although, typically associated with primary sclerosing cholangitis, the presence of inflammatory bowel disease (IBD; both Crohn's disease and ulcerative colitis) has been described in 2-8% of patients with AIH. Interestingly, for patients with autoimmune sclerosing cholangitis, a distinct pattern of IBD has been recently described. Other conditions have been reported at a lower frequency, including Sjogren's syndrome 1-7%, systemic lupus erythematosus 1-3% and glomerulonephritis 1%. Rarer still and at a frequency of <1% include fibrosing alveolitis, haemolytic anaemia, uveitis, mononeuritis multiplex, polymyositis and multiple sclerosis. In contrast, the reported associations between AIH and thyroiditis 8-23%, diabetes 1-10% and psoriasis 3% are commonly seen and notable in clinical practice.

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Section: Effects Of Aih Types Age Gender and Genetic Backgroundmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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“…DNA sequence analysis of DQ and DR genes from white patients with insulin-dependent diabetes mellitus and rheumatoid arthritis indicates that particular amino acid sequences in the hypervariable regions of DR or DQ beta alleles determine both susceptibility and resistance to diseases (20,21). For autoimmune hepatitis among white persons, the primary association with the major histocompatibility complex at the functional level seems to involve the class 11, e.g., DR3 or other class I1 alleles in linkage disequilibrium with DR3, such as DRw52 and DQw2 (22). To date no informative disease-associated restriction fragment length polymorphisms have been identified to be associated with autoimmune hepatitis among white patients (12).…”

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confidence: 99%

Association of autoimmune hepatitis with HLA-Bw54 and DR4 in Japanese patients

et al. 1990

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Human leukocyte antigen-D region-related alleles (human leukocyte antigen DR and DQ) and human leukocyte antigen class I alleles were typed serologically in 31 Japanese patients with autoimmune hepatitis. These patients had increased serum levels of AST and IgG, high titers of autoantibodies, no history of blood transfusion and were negative for HBsAg and antibodies to HBc. Three hundred eighty-six healthy subjects and 30 patients with cryptogenic chronic hepatitis served as control groups. The frequency of DR4 was significantly higher in autoimmune hepatitis patients (90.3%) than in healthy subjects (38.6%) and in cryptogenic chronic hepatitis patients (30%). The frequency of Bw54 was significantly higher in autoimmune hepatitis patients (45.2%) than in healthy subjects (10.9%). The risk to DR4-positive subjects for autoimmune hepatitis was 14.8 relative to healthy subjects. Two of 31 patients (6.5%) with autoimmune hepatitis were positive for antibody to hepatitis C virus; both clearly satisfied criteria for autoimmune hepatitis and both had Bw54 and DR4. This study revealed a highly significant association of autoimmune hepatitis with human leukocyte antigen Bw54 and DR4 in Japanese patients. Among the DR4-positive patients with autoimmune hepatitis, no significant differences were seen between those positive or negative for Bw54 with regard to clinical or laboratory data, relapse of disease or efficacy of prednisolone. Thus human leukocyte antigen class II alleles contribute to susceptibility and resistance to autoimmune hepatitis in Japanese patients, with distinct racial differences from those in white patients.

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“…AIH flares are characterized by high frequencies of both Tregs and effector T cells (Teffs) in the inflamed liver [8,9] (Fig. The current study reports that steroid and azathioprine therapy has a greater effect on intrahepatic Tregs compared with T effector cells [1,9]. Now Taubert and colleagues show that patients reaching biochemical remission have higher intrahepatic Treg/Teff and Treg/B cell ratios compared to those who fail to respond.…”

mentioning

confidence: 66%

“…Although the aetiology is still unknown the development of the disease involves a loss of immunological self-tolerance to liver antigens resulting in a T cell mediated destruction of hepatocytes [1]. It is more commonly seen in women, strongly associated with variants in the major histocompatibility complex region and usually responsive to immunosuppression.…”

mentioning

confidence: 99%

Regulatory T cells and autoimmune hepatitis: What happens in the liver stays in the liver

Adams

2014

Journal of Hepatology

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HLA A1, B8, DR3 extended haplotypes in autoimmune chronic hepatitis

Cited by 32 publications

References 9 publications

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Association of autoimmune hepatitis with HLA-Bw54 and DR4 in Japanese patients

Regulatory T cells and autoimmune hepatitis: What happens in the liver stays in the liver

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