Clinical and HLA phenotypes of type 1 autoimmune hepatitis in North American patients outside DR3 and DR4

: Aims: To determine the clinical phenotype and outcome of patients with definite type 1 autoimmune hepatitis, who lack human leukocyte antigen (HLA) DR3 and DR4, and to assess the importance of HLA DR7 and DR13. Methods: Two hundred and seven adult patients were typed for DR3, DR4, DR7, and DR13 by...

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Bibliographic Details
Published in:Liver international 2006-06, Vol.26 (5), p.552-558
Main Authors: Czaja, Albert J., Carpenter, Herschel A., Moore, S. Breanndan
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - therapeutic use
Adult
autoimmune hepatitis
autoimmunity
Disease Susceptibility
Female
genetic predispositions
Hepatitis, Autoimmune - drug therapy
Hepatitis, Autoimmune - epidemiology
Hepatitis, Autoimmune - immunology
HLA
HLA Antigens - analysis
HLA-DR Antigens - analysis
HLA-DR3 Antigen
HLA-DR4 Antigen
HLA-DR7 Antigen
HLA-DRB1 Chains
Humans
Immunophenotyping
Male
Middle Aged
North America - epidemiology
Recurrence
Treatment Failure
Treatment Outcome
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Summary:: Aims: To determine the clinical phenotype and outcome of patients with definite type 1 autoimmune hepatitis, who lack human leukocyte antigen (HLA) DR3 and DR4, and to assess the importance of HLA DR7 and DR13. Methods: Two hundred and seven adult patients were typed for DR3, DR4, DR7, and DR13 by DNA‐based techniques. One hundred and two blood donors constituted a normal population. Results: Twenty‐six patients lacked DR3 and DR4 (13%). Treatment failure occurred more commonly in these individuals than in the 68 patients with DR4 (20% vs. 3%, P=0.03), and relapse after drug withdrawal was less frequent than in the 84 patients with DR3 (55% vs. 87%, P=0.03). HLA DR13 occurred more often than in those with DR3 (54% vs. 15%, P=0.0002) or DR4 (54% vs. 12%, P=0.00005), and it was more frequent than in normal adults (54% vs. 22%, P=0.003), including those without DR3 or DR4 (54% vs. 27%, P=0.03). HLA DR7 was not associated with susceptibility or outcome. Conclusions: White North American patients who lack DR3 and DR4 respond differently to corticosteroid treatment than patients with classical HLA phenotypes. HLA DR13 is common in these adult patients, and it may affect treatment outcome.
ISSN:1478-3223
1478-3231
DOI:10.1111/j.1478-3231.2006.01249.x