title: Correlation of clinical severity and ELISA indices for the NC16A domain of BP180 measured using BP180 ELISA kit in bullous pemphigoid.
abstract: BACKGROUND
  Titres of circulating autoantibodies detected by indirect immunofluorescence (IIF) have been used for the diagnosis and evaluation of disease activity in bullous pemphigoid (BP). In BP, the major pathogenic epitope is known to be the non-collagenous extracellular domain (NC16A) of the 180-kDa transmembrane hemidesmosomal protein (BPAG2). Recently, an enzyme-linked immunosorbent assay (ELISA) kit using the NC16A domain recombinant protein (BP180 ELISA kit) has become commercially available to measure the quantities of pathogenic autoantibodies circulating in BP patients.
  
  
  OBJECTIVE
  To investigate the correlation of clinical severity and ELISA indices in BP.
  
  
  METHODS
  Fourteen patients with a typical form of BP and one refractory BP patient who died despite extensive treatment were included in this study. Antibody titres in sera from these patients were measured using BP180 ELISA kit and an analysis of ELISA indices with disease activity was performed.
  
  
  RESULTS
  ELISA indices were significantly reduced after successful therapy, although IIF titres did not always show apparent correlations. In the patient with refractory BP, ELISA indices also showed a good correlation with disease course. ELISA indices measured using the BP180 ELISA kit were well correlated with the disease activity.
  
  
  CONCLUSIONS
  This commercially available kit more closely followed disease activities than the IIF titres. The BP ELISA system may be a useful tool to evaluate the disease activity and to assess the effectiveness of the treatment of BP.
authors.affiliation: Department of Dermatology, Hokkaido University Graduate School of Medicine, Kita-ku, Sapporo 060-8638, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Tsuji-Abe
authors.forename: Masashi
authors.initials: M
authors.lastname: Akiyama
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Yamanaka
authors.forename: Toshiro
authors.initials: T
authors.lastname: Kikuchi
authors.forename: Kazuko C
authors.initials: KC
authors.lastname: Sato-Matsumura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Shimizu
mesh_terms: D001324:Autoantigens / Q000032:analysis / Q000737:chemistry* / Q000276:immunology*; D002624:Chemistry, Clinical / Q000379:methods*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods*; D000939:Epitopes; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008297:Male; D008875:Middle Aged; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000175:diagnosis* / Q000378:metabolism; D017434:Protein Structure, Tertiary; D011994:Recombinant Proteins / Q000737:chemistry; D012074:Remission Induction; D013997:Time Factors; D000097805:Collagen Type XVII
chemical_list: D001324:Autoantigens; D000939:Epitopes; D024041:Non-Fibrillar Collagens; D011994:Recombinant Proteins; D000097805:Collagen Type XVII