Cargando…

The relationship between HLA-B*51 subtypes, clinical manifestations and severity of Behçet’s syndrome: a large Italian cohort study

OBJECTIVES: Behçet’s syndrome (BS) is a chronic multisystemic inflammatory disorder of unclear aetiology. The predominant BS susceptibility locus was identified within HLA-B*51. HLA-B*51 subtypes were previously studied as disease susceptibility markers. Few data are now available about the relation...

Descripción completa

Detalles Bibliográficos
Autores principales: Leccese, Pietro, Padula, Maria Carmela, Santospirito, Eustachio Vincenzo, Colucci, Rosa, Lascaro, Nancy, Padula, Angela Anna, D’Angelo, Salvatore
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10627283/
https://www.ncbi.nlm.nih.gov/pubmed/37937176
http://dx.doi.org/10.1093/rap/rkad087
Descripción
Sumario:OBJECTIVES: Behçet’s syndrome (BS) is a chronic multisystemic inflammatory disorder of unclear aetiology. The predominant BS susceptibility locus was identified within HLA-B*51. HLA-B*51 subtypes were previously studied as disease susceptibility markers. Few data are now available about the relationship between B*51 subtypes and clinical phenotype. The aim of this study was to genotype HLA-B*51 subtypes in a series of Italian BS patients and to test the association with clinical manifestations and disease severity (Krause’s index). METHODS: HLA-B*51 subtype genotyping for 63 alleles (B*51:01–B*51:63) was performed by PCR after DNA extraction from whole blood of BS patients. The correlation of disease clinical manifestations and severity (Krause’s index) with the HLA-B*51 allele and its subtypes was analysed. RESULTS: We enrolled 241 (140 male and 101 female) BS patients, and HLA-B*51 frequency was 62.7% (151 of 241). One hundred and eight of the HLA-B*51-positive patients carried the B*51:01 subtype (108 of 151, 71.5%), 39 of 151 (25.8%) the B*51:08 subtype, 2 of 151 (1.3%) the B*51:02 subtype, 1 of 151 (0.7%) the B*51:05 subtype, and 1 of 151 (0.7%) the B*51:07 subtype. We found that ocular involvement was statistically associated with HLA-B*51 positivity and with B*51:01 and B*51:08 subtypes (P < 0.05). We also found that disease severity was higher in HLA-B*51-positive patients than in negative patients, but without statistical significance (median Krause’s index 5.1 vs 4.1, P > 0.05). CONCLUSION: Here, we confirm a high frequency of the HLA-B*51 allele in our group of BS patients. B*51:01 and B*51:08 were found to be the most common subtypes, and an association of both subtypes with ocular involvement was also underlined.