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Behçet’s Disease as a Canker Sore: MHC-I-Opathy Versus Behcet’s Spectrum Disorders

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Abstract

Purpose of Review

Behçet’s disease is a chronic multisystemic inflammatory disorder characterized by recurrent oral ulcers and a close association with HLA-B*51. This review summarizes the clinical and genetic features of Behçet’s disease and compares susceptibility genes with those of other HLA class I-associated and stomatitis-related diseases.

Recent Findings

In Behçet’s disease, recently identified non-HLA susceptibility genes are involved in the innate and acquired immune functions. An epistatic interaction between HLA-B*51 and ERAP1 is considered to play a pathogenic role in the disease. Similar findings have been also shown in other HLA class I-associated diseases, leading to a new concept of MHC-I-opathy.

Immune-related non-HLA susceptibility genes are shared among Behçet’s disease, recurrent aphthous stomatitis, and periodic fever aphthous stomatitis and adenitis syndrome, leading to another novel concept of Behçet’s spectrum disorders.

Summary

Recent genetic studies have shown that Behçet’s disease has both features of MHC-I-opathy and Behçet’s spectrum disorders.

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Funding

This study was supported by grants from the Japanese Society for the Promotion of Science Grants-in-Aid for Scientific Research # 21K08467 and Behçet’s Disease Research Committee, Health Labor Sciences Research Grant (23FC1020) for the author (M. Takeno).

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  1. Department of Allergy and Rheumatology, Nippon Medical School, Musashi Kosugi Hospital, 1-383 Kosugi-machi, Nakahara-ku, Kawasaki, Kanagawa, 211-8533, Japan

    Mitsuhiro Takeno

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Correspondence to Mitsuhiro Takeno.

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Takeno, M. Behçet’s Disease as a Canker Sore: MHC-I-Opathy Versus Behcet’s Spectrum Disorders. Curr Oral Health Rep 11, 23–29 (2024). https://doi.org/10.1007/s40496-024-00362-7

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