Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity

• DOI: 10.1111/j.1365-2133.2005.06998.x
• Publication Date: 2005-11-18T23:36:49Z
• Authors: N. Oyama; J.F. Setterfield; A.M. Powell; Y. Sakuma-Oyama; S. Albert; B.S. Bhogal; R.W. Vaughan; F. Kaneko; S.J. Challacombe; M.M. Black
• Keywords: Adult; Male; Dystonin; Genes, MHC Class II; Immunoblotting; 610; Autoantigens; Basement Membrane; 03 medical and health sciences; 0302 clinical medicine; Humans; Alleles; Aged; Autoantibodies; Aged, 80 and over; Middle Aged; Immunoglobulin A; 3. Good health; Cytoskeletal Proteins; Microscopy, Fluorescence; Immunoglobulin G; Female; Laminin; Carrier Proteins

Background Mucous membrane pemphigoid (MMP), a chronic autoimmune subepithelial blistering disease, is associated with circulating IgG and/or IgA autoantibodies against several basement zone antigens. The heterogeneity of clinical presentation and diversity target autoantigens have contributed to difficulties in characterizing this condition immunologically. Objectives To analyse serum autoantibody profile HLA class II alleles MMP patients correlate the disease. Methods Well‐defined subgroups consisting 124 were examined for reactivity immunoblotting using human epidermal, dermal placental amnion proteins. results further analysed on basis detailed (sites involvement disease severity) immunopathological criteria (immunofluorescence study alleles). Results Immunoblot assay revealed that majority had (93 124, 75%) (63 51%) BP180 (including its soluble ectodomains, 120‐kDa LAD‐1 97‐kDa LABD97 antigens). Other antigens targeted predominantly by included: BP230 34 (27%), β4 integrin 26 (21%), laminin 5 three (2%). All BP230+ sera 23 (88%) integrin+ also reacted at least one Over 85% ocular involvement. In most cases MMP, more severe features antibody multiple antigens, as well component Dual BP180/LAD‐1 was phenotype. addition, subset‐dependent correlated specific alleles, DQB1*0301, DRB1*04 DRB1*11. Conclusions Our confirmed major autoantigen MMP. disease‐prevalent humoral response particular subsets antigenic epitope(s) within ectodomain may contribute clinicopathological significance severity