title: Prevalence and clinical significance of anti-laminin 332 autoantibodies detected by a novel enzyme-linked immunosorbent assay in mucous membrane pemphigoid.
abstract: OBJECTIVE
  A rare variant of mucous membrane pemphigoid (MMP) is characterized by circulating anti-laminin 332 (Lam332) autoantibodies and seems to be associated with concurrent malignant neoplasms.
  
  
  OBJECTIVE
  To determine the prevalence and clinical significance of anti-Lam332 autoantibody detection from a large series of patients with MMP.
  
  
  METHODS
  Multicenter retrospective study.
  
  
  METHODS
  Four French national centers for autoimmune bullous diseases.
  
  
  METHODS
  One hundred fifty-four patients with MMP and 89 individuals serving as controls were included.
  
  
  METHODS
  Serum samples were analyzed by a new Lam332 enzyme-linked immunosorbent assay (ELISA); clinical and immunopathologic data were obtained from the patients' medical records.
  
  
  METHODS
  The Lam332 ELISA scores were evaluated with respect to clinical characteristics, standard and salt-split indirect immunofluorescence, and bullous pemphigoid (BP) 230 and BP180-NC16A ELISAs.
  
  
  RESULTS
  The Lam332 ELISA score was positive (≥9 U/mL) in 20.1% of serum samples from patients with MMP, 1 of 50 patients with bullous pemphigoid (BP), none of 7 with pemphigus, and 3 of 32 other controls. No relationship was evidenced between a positive ELISA Lam332 score and age; sex ratio; oral, ocular, genital, skin, or esophageal/laryngeal involvement; internal malignant neoplasm; or BP180 ELISA score. Salt-split skin indirect immunofluorescence and ELISA BP230 results were more frequently positive when Lam332 ELISA results were positive (P = .04 and .02, respectively). Patients with a positive Lam332 ELISA score frequently had more severe MMP (67.8% vs 47.2%; P = .04).
  
  
  CONCLUSIONS
  Results of this novel ELISA showed that serum anti-Lam332 autoantibodies are detected in 20.1% of patients with MMP. Anti-Lam332 autoantibodies are mainly detected in patients with severe MMP but not preferentially in those with a malignant neoplasm. The association between anti-Lam332 and anti-BP230 autoantibodies might arise from an epitope-spreading phenomenon.
authors: Department of Dermatology, Reims University Hospital, University of Reims-Champagne-Ardenne, Reims, France. pbernard@chu-reims.fr
authors: Philippe
authors: P
authors: Bernard
authors: Frank
authors: F
authors: Antonicelli
authors: Christophe
authors: C
authors: Bedane
authors: Pascal
authors: P
authors: Joly
authors: Christelle
authors: C
authors: Le Roux-Villet
authors: Sophie
authors: S
authors: Duvert-Lehembre
authors: Patricia
authors: P
authors: Rousselle
authors: Catherine
authors: C
authors: Prost-Squarcioni
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood* / Q000302:isolation & purification; D002352:Carrier Proteins; D016022:Case-Control Studies; D015815:Cell Adhesion Molecules / Q000276:immunology*; D016009:Chi-Square Distribution; D002648:Child; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000276:immunology; D008875:Middle Aged; D009369:Neoplasms / Q000097:blood*; D009419:Nerve Tissue Proteins; D010390:Pemphigoid, Benign Mucous Membrane / Q000097:blood*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D055815:Young Adult; D000097794:Kalinin
chemical_list: D001323:Autoantibodies; D002352:Carrier Proteins; D015815:Cell Adhesion Molecules; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D008562:Membrane Glycoproteins; D009419:Nerve Tissue Proteins; D000097794:Kalinin; C486268:DST protein, human