Mucous membrane pemphigoid: HLA-DQB1*0301 is associated with all clinical sites of involvement and may be linked to antibasement membrane IgG production

摘要: Background  Class I human leucocyte antigens (HLA) -A, -B, -Cw and class II HLA-DRB1, -DQB1 alleles were determined in 131 British Caucasian patients with mucous membrane pemphigoid (MMP) using serological DNA-based methods. Objectives  To analyse the expressed well-defined clinical immunopathological subgroups of MMP, order to establish whether specific or haplotypes might part explain disease susceptibility, sites involvement severity. Methods  Subgroups analysed according following criteria: age onset, sex, (oral, ocular, skin, nasal, genital, pharyngeal, oesophageal, laryngeal, perianal), severity history autoimmune disease. Subgroups also autoantibody profile, presence circulating antibasement IgG IgA antibodies detection target basement zone (BMZ) (BP230 BP180) by autoantibodies. Results HLA typing showed no significant subgroup associations. Class DRB1 a significantly increased allelic frequency MMP vs. controls for DRB1*11 (RR = 2·08, Pc < 0·0000056). For DQB1, controls, there was DQB1*0301 (Pc < 0·00000028) both males females; all involvement, exception oesophageal perianal detectable anti-BMZ compared those negative (P < 0·0096, Pc < 0·019). A positive trend noted ocular score  10 < 10. We found difference allele between without BP180 BP230 antigens. Haplotype frequencies an increase DRB1*04, (Pc < 0·000066) DRB1*11, (Pc < 0·000002) among controls. Conclusions  The confers predisposition may have role T-cell recognition antigens, resulting production autoantibodies. The expression higher score, further suggests this severity.