Distribution of HLA class II alleles among Spanish patients with pemphigus vulgaris

Abstract

Twenty-six unrelated Spanish Caucasian individuals affected by pemphigus vulgaris (PV) were HLA typed and frequencies compared with those of 200 ethnically matched healthy controls. Twenty-three out of 26 patients were HLA-DR4. The frequency of HLA-DR14 was also increased (31%; controls: 4%). Of the 23 patients positive for HLA-DR4, 21 carried the DRB1*0402 allele. Therefore, the frequency of HLA-DRB1*0402 among patients was 81% (4% in controls; P=4.7x10(-27), OR=100.8). Interestingly, HLA-DR13, a frequent HLA-DR specificity in the Spanish general population (27%), was absent among the PV patients (P=0.009; Pc=0.1; OR=0.05). Taking together these data, we can conclude that, in the Spanish population, PV is preferentially and strongly associated with HLA-DRB1*0402, whereas DRB1*13 seems to confer a protective effect in our population.

HLA Class II Polymorphism Contributes to Specify Desmoglein Derived Peptides in Pemphigus Vulgaris and Pemphigus Foliaceus

“…Our results confirm previous reports showing a strong association of PV with DQB1*0503 which is in linkage disequilibrium with DR14 alleles [3,4,5,8,10,12,13,14,15,16,17]. Since the DQB1*0503 PV patients had various DRB1*14 related alleles, one alternative The AA residues are shown using the letter code.…”

“…The increased frequency of DRB1*0402 corroborates previous studies in Ashkenazi Jewish patients [2] and in non-Jewish Caucasians [3,4,5,6,10]. The DRB1*1401 or 1404 alleles were found associated with PV in non-Jewish patients of European ancestry (Italy, Sardinia, Spain, Boston area) [3,4,5,6] and in Asian populations (India, Pakistan, Japan) [12,13,14,15,16,17].…”

“…Endemic and non-endemic PF have been described with a predominance of endemic PF in Brazil and in NorthAfrica (Tunisia). A strong association with MHC molecules has already been shown in PV (DRB1*0402 and DQB1*0503) [2,3,4,5,6] and in endemic PF (DRB1*0102, 04040, 1402 or 1406) [7,8,9]. The HLA susceptibility to PV has not been determined in France, and previous studies (with the exception of a recent study of Italian patients) [10] concerning nonendemic PF were carried out on small patient groups and were therefore inconclusive.…”

“…The DRB1*1401 or 1404 alleles were found associated with PV in non-Jewish patients of European ancestry (Italy, Sardinia, Spain, Boston area) [3,4,5,6] and in Asian populations (India, Pakistan, Japan) [12,13,14,15,16,17].…”

Association of human leukocyte antigen class II alleles with pemphigus vulgaris in a Turkish population

“…Gonzá lez-Escribano et al 16 found that among Spanish patients with PV, HLA DR4 allele was high and HLA DR14 frequency was also increased. Interestingly, HLA DR13, a frequent HLA DR specificity in the Spanish general population, was absent among the PV patients.…”