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Association of DR4 with pemphigus
A R Ahmed1, M S Park, J L Tiwari
1Center for Blood Research, Harvard University, Boston, Mass.
Experimental and Clinical Immunogenetics
|January 1, 1987
Summary
Pemphigus vulgaris is strongly associated with specific Human Leukocyte Antigen (HLA) types, particularly HLA-DR4. This association appears to correlate with disease severity, especially in patients with higher antibody titers.
Area of Science:
- Immunogenetics
- Dermatology
- Autoimmune Diseases
Background:
- Pemphigus vulgaris is a rare autoimmune blistering disease.
- Human Leukocyte Antigen (HLA) genes are crucial for immune regulation and have been implicated in various autoimmune conditions.
Purpose of the Study:
- To investigate the association between specific HLA antigens and pemphigus vulgaris in a Los Angeles population.
- To explore correlations between HLA antigen presence, disease characteristics, and antibody titers.
Main Methods:
- HLA typing (A, B, C, and D loci) was performed on 65 pemphigus vulgaris patients and 558 controls.
- Patients were categorized by ethnicity, disease manifestation, disease course, and anti-intercellular substance antibody titers.
Main Results:
- A statistically significant increase in HLA-A25, HLA-B38, and HLA-DR4 antigens was observed in patients compared to controls.
- The association was more pronounced in Jewish patients and correlated with higher antibody titers, suggesting a link to disease severity.
- HLA-DR4 showed the strongest association, independent of HLA-B38 presence.
Conclusions:
- The Human Leukocyte Antigen (HLA)-DR4 antigen is strongly associated with pemphigus vulgaris.
- HLA-DR4 may serve as a marker for disease severity in pemphigus vulgaris, particularly in individuals with elevated antibody levels.