title: Prospective studies on the routine use of a novel multivariant enzyme-linked immunosorbent assay for the diagnosis of autoimmune bullous diseases.
abstract: BACKGROUND
  Serologic diagnosis of autoimmune blistering disease (AIBD) usually follows a sophisticated multistep algorithm.
  
  
  OBJECTIVE
  We sought validation of a multivariant enzyme-linked immunosorbent assay (ELISA) in the routine diagnosis of AIBD.
  
  
  METHODS
  The multivariant ELISA comprising 6 recombinant immunodominant forms of major AIBD target antigens, ie, desmoglein 1, desmoglein 3, envoplakin, BP180, BP230, and type VII collagen was applied in: (1) a cohort of well-characterized AIBD (n = 173) and control sera (n = 130), (2) a prospective multicenter study with 204 sera from patients with newly diagnosed AIBD with positive direct immunofluorescence microscopy, and (3) a prospective monocenter study with 292 consecutive sera from patients with clinical suspicion of AIBD in comparison with the conventional multistep diagnostic algorithm.
  
  
  RESULTS
  Concordant results in the multivariant ELISA compared with direct immunofluorescence microscopy were seen in 94% of patients with pemphigus and 71% of patients with pemphigoid (Cohen κ value, 0.95 and 0.66) and with the conventional multistep diagnostic approach in 91% of patients with pemphigus and 88% of patients with bullous pemphigoid and 93% of autoantibody-negative sera (Cohen κ, 0.95, 0.84, and 0.78).
  
  
  CONCLUSIONS
  IgA autoantibodies and less common target antigens were not analyzed.
  
  
  CONCLUSIONS
  The multivariant ELISA is a practical, highly standardized, and widely available novel diagnostic tool for the routine diagnosis of AIBD.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Nina
authors.initials: N
authors.lastname: van Beek
authors.affiliation: Institute of Experimental Immunology, Euroimmun, Lübeck, Germany.
authors.forename: Cornelia
authors.initials: C
authors.lastname: Dähnrich
authors.affiliation: Institute of Experimental Immunology, Euroimmun, Lübeck, Germany.
authors.forename: Nora
authors.initials: N
authors.lastname: Johannsen
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany; Human Immunophenotyping Laboratory, University of Lübeck, Lübeck, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Lemcke
authors.affiliation: Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Goletz
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Franziska
authors.initials: F
authors.lastname: Hübner
authors.affiliation: Molecular and Cell Biology Laboratory, Rome, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Zenzo
authors.affiliation: Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.
authors.forename: Marian
authors.initials: M
authors.lastname: Dmochowski
authors.affiliation: Department of Dermatology and Venereology, Medical Faculty of Medicine, Sofia, Bulgaria.
authors.forename: Kossara
authors.initials: K
authors.lastname: Drenovska
authors.affiliation: Dermatology Department Tel Aviv, Sourasky Medical Center, Tel Aviv, Israel.
authors.forename: Shamir
authors.initials: S
authors.lastname: Geller
authors.affiliation: University Institute of Clinical Chemistry and Center of Laboratory Medicine, Inselspital, Bern, Switzerland.
authors.forename: Michael
authors.initials: M
authors.lastname: Horn
authors.affiliation: Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Cezary
authors.initials: C
authors.lastname: Kowalewski
authors.affiliation: Department of Dermatology, University of Belgrade, School of Medicine Pasterova 2, Belgrade, Serbia.
authors.forename: Ljiljana
authors.initials: L
authors.lastname: Medenica
authors.affiliation: Department of Dermatology St George Hospital, University of New South Wales School of Medicine, Sydney, Australia.
authors.forename: Dedee F
authors.initials: DF
authors.lastname: Murrell
authors.affiliation: Second University Dermatology Department, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece.
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Patsatsi
authors.affiliation: Akdeniz University Faculty of Medicine Department of Dermatology, Antalya, Turkey.
authors.forename: Soner
authors.initials: S
authors.lastname: Uzun
authors.affiliation: Department of Dermatology and Venereology, Medical Faculty of Medicine, Sofia, Bulgaria.
authors.forename: Snejina
authors.initials: S
authors.lastname: Vassileva
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Human Immunophenotyping Laboratory, University of Lübeck, Lübeck, Germany.
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.affiliation: Institute of Experimental Immunology, Euroimmun, Lübeck, Germany.
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Schlumberger
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany. Electronic address: enno.schmidt@uksh.de.
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
mesh_terms: D000465:Algorithms; D001324:Autoantigens / Q000097:blood; D001327:Autoimmune Diseases / Q000097:blood* / Q000175:diagnosis*; D024143:Collagen Type VII / Q000097:blood; D051183:Desmoglein 1 / Q000097:blood; D051184:Desmoglein 3 / Q000097:blood; D000071821:Dystonin / Q000097:blood; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D008565:Membrane Proteins / Q000097:blood; D008853:Microscopy; D024041:Non-Fibrillar Collagens / Q000097:blood; D011446:Prospective Studies; D011498:Protein Precursors / Q000097:blood; D012372:ROC Curve; D011994:Recombinant Proteins / Q000276:immunology; D012872:Skin Diseases, Vesiculobullous / Q000097:blood* / Q000175:diagnosis*; D000097805:Collagen Type XVII
chemical_list: D001324:Autoantigens; D024143:Collagen Type VII; D051183:Desmoglein 1; D051184:Desmoglein 3; D000071821:Dystonin; D008565:Membrane Proteins; D024041:Non-Fibrillar Collagens; D011498:Protein Precursors; D011994:Recombinant Proteins; D000097805:Collagen Type XVII; C486268:DST protein, human; C100754:envoplakin
keywords: autoantibody; blistering; bullous pemphigoid; epidermolysis bullosa acquisita; pemphigus