title: BP230- and BP180-specific auto-antibodies in bullous pemphigoid.
abstract: Bullous pemphigoid is a subepidermal blistering disease associated with auto-antibodies (auto-ab) to BP180 and BP230. We developed ELISAs utilizing baculovirus-encoded recombinant proteins of BP230 and BP180 and studied their diagnostic and prognostic values by assessing the profile of the auto-ab response in 127 patients with BP. 39 patients had focal involvement, whereas 88 had generalized disease; 51 individuals served as controls. The results indicate: (1) BP180 IgG reactivity was associated with an overall sensitivity of 0.953 and specificity of 0.940; (2) 105 of 127 BP patients also displayed BP230 auto-reactivity, the global diagnostic performance of which, however, was moderate compared to BP180-auto-reactivity (sensitivity 0.815 vs 0.953, specificity 0.648 vs 0.940); (3) 101 patients (79.5%) had concordant BP180 and BP230 reactivity; (4) the association between the presence of BP230 auto-reactivity and focal involvement was stronger than in generalized disease (odds ratio (OR) 17.7 vs 10.2), independently from BP180 auto-ab profile; (5) correlation of total IgG with IgG1 and IgG4 was variable for both BP180 and BP230. Collectively, the global diagnostic properties of the BP180-ELISA outperform those of the BP230-ELISA. Presence of BP230 auto-reactivity, however, supports the diagnosis of BP and might be indicative for the extent of the disease.
authors.affiliation: Department of Dermatology, Friedrich-Alexander-University, Erlangen-Nürnberg, Germany. sybille.thoma-uszynski@derma.imed.uni-erlangen.de
authors.forename: Sybille
authors.initials: S
authors.lastname: Thoma-Uszynski
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Uter
authors.forename: Susanne
authors.initials: S
authors.lastname: Schwietzke
authors.forename: Silke C
authors.initials: SC
authors.lastname: Hofmann
authors.forename: Thomas
authors.initials: T
authors.lastname: Hunziker
authors.forename: Philippe
authors.initials: P
authors.lastname: Bernard
authors.forename: Regina
authors.initials: R
authors.lastname: Treudler
authors.forename: Christos C
authors.initials: CC
authors.lastname: Zouboulis
authors.forename: Gerold
authors.initials: G
authors.lastname: Schuler
authors.forename: Luca
authors.initials: L
authors.lastname: Borradori
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000276:immunology; D001324:Autoantigens / Q000737:chemistry / Q000276:immunology*; D016367:Baculoviridae / Q000235:genetics; D003094:Collagen / Q000737:chemistry / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male; D008875:Middle Aged; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000276:immunology*; D017434:Protein Structure, Tertiary; D011994:Recombinant Proteins; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D003094:Collagen; D007074:Immunoglobulin G; D024041:Non-Fibrillar Collagens; D011994:Recombinant Proteins; D000097805:Collagen Type XVII