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Human leukocyte antigen DR status and clinical features in Japanese patients with type 1 autoimmune hepatitis
Yasuhiro Miyake1, Yoshiaki Iwasaki, Akinobu Takaki
1Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
Insights
Human leukocyte antigen (HLA) DR status influences autoimmune hepatitis in Japan. DR2 and DR4 are common, unlike DR3, and associated with specific clinical features, impacting diagnosis in younger patients.
Area of Science:
- Immunogenetics
- Hepatology
- Autoimmune Diseases
Background:
- Human leukocyte antigen (HLA) DR status is known to influence autoimmune hepatitis (AIH) clinical presentation and outcomes, particularly in Caucasian populations where DR3 is associated with poorer prognosis.
- The specific relationship between HLA DR status and the clinical features of type 1 AIH in Japanese patients remains largely unexamined.
Purpose of the Study:
- To investigate the association between HLA DR status and clinical features in Japanese patients with type 1 autoimmune hepatitis.
- To determine if specific HLA DR alleles correlate with disease presentation, immunoglobulin levels, concurrent autoimmune diseases, or treatment response in this population.
Main Methods:
- A cohort of 79 Japanese patients diagnosed with type 1 autoimmune hepatitis was studied.
- HLA DR typing was performed using the polymerase chain reaction sequence specific oligonucleotide hybridization method.
- Clinical data, including age, immunoglobulin G levels, concurrent autoimmune diseases, and treatment response, were analyzed in relation to HLA DR status.
Main Results:
- The study found DR4 (55 patients) and DR2 (23 patients) to be the predominant HLA DR types, with no patients positive for DR3.
- Patients with DR4 showed a tendency towards higher immunoglobulin G levels, while those with neither DR2 nor DR4 had lower levels.
- DR2 was associated with a lower frequency of concurrent autoimmune diseases, and thyroid disease was exclusively observed in DR4 patients. Treatment response was not linked to HLA DR status.
Conclusions:
- HLA DR status significantly influences the clinical characteristics of type 1 autoimmune hepatitis in Japanese patients.
- Japanese patients with HLA DR2 may exhibit distinct clinical features compared to other patient groups.
- Careful consideration of HLA DR status (particularly the absence of DR2/DR4) and age (<30 years) is crucial for accurate diagnosis of type 1 AIH in Japanese individuals.
Aim:
Human leukocyte antigen (HLA) DR status affects the clinical features of autoimmune hepatitis. In Caucasians, patients with DR3 have poorer outcomes. In Japan, the relationship between HLA DR status and clinical features has yet to be fully examined.
Methods:
We investigated 79 patients with type 1 autoimmune hepatitis who underwent liver biopsy and were screened for HLA DR status by the polymerase chain reaction sequence specific oligonucleotide hybridization method.
Results:
Fifty-five patients had DR4 and 23 had DR2. Thirteen patients had both DR2 and DR4. None had DR3. Of patients aged <30 years, 70% did not have DR4. A tendency toward higher serum levels of immunoglobulin G was seen in patients with DR4 compared to those without, while patients with neither DR2 nor DR4 had lower serum levels of immunoglobulin G than those with only DR2 and those with only DR4. Patients with DR2 had a lower frequency of concurrentautoimmune disease. Concurrence of thyroid disease was seen only in patients with DR4. The cumulative incidental rate of the normalization of serum alanine aminotransferase levels within six months after the introduction of corticosteroid treatment was not associated with HLA DR status.
Conclusion:
HLA DR status is considered to affect the clinical features of Japanese patients with type 1 autoimmune hepatitis. Japanese patients with DR2 may have different clinical features from others. In addition, diagnoses of type 1 autoimmune hepatitis should be made carefully in Japanese patients with neither DR2 nor DR4 and in those aged <30 years.
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