title: Increased serum levels of circulating CD40 ligand in patients with bullous pemphigoid: preliminary results.
abstract: BACKGROUND
  Autoimmune bullous diseases are characterized by autoantibodies against specific adhesion molecules of the skin and/or mucous membrane. While these autoantibodies are known to play a primary role in the disease manifestation, it remains unknown how disease-specific autoreactive B cells and autoantibodies are induced. Recent studies have indicated the importance of the CD40 and CD40 ligand (CD40L) receptor-ligand pair in the immunopathogenesis of autoimmune diseases. CD40L circulates in soluble form, and some reports suggest that serum soluble CD40L (sCD40L) levels are increased in various autoimmune diseases.
  
  
  OBJECTIVE
  To determine serum sCD40L levels in patients with pemphigus vulgaris (PV) and bullous pemphigoid (BP), and to determine their correlation with clinical findings and laboratory findings.
  
  
  METHODS
  Sera from 10 PV patients, 35 BP patients and 12 normal controls were subjected to ELISA assays to measure serum levels of sCD40L, anti-desmoglein-3 antibody and anti-BP180 antibody.
  
  
  CONCLUSIONS
  Circulating sCD40L levels were significantly elevated in BP patients, but not in PV patients. Serum sCD40L levels increased in the early stage of disease onset and recurrence in BP patients. In conclusion, circulating sCD40L levels may be a useful marker for early activation of autoimmune diathesis and, furthermore, an effective therapeutic target in patients with BP.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Watanabe
authors.forename: N
authors.initials: N
authors.lastname: Ishiura
authors.forename: H
authors.initials: H
authors.lastname: Nakashima
authors.forename: N
authors.initials: N
authors.lastname: Yazawa
authors.forename: Y
authors.initials: Y
authors.lastname: Kuwano
authors.forename: Y
authors.initials: Y
authors.lastname: Tada
authors.forename: H
authors.initials: H
authors.lastname: Okochi
authors.forename: M
authors.initials: M
authors.lastname: Fujimoto
authors.forename: K
authors.initials: K
authors.lastname: Tamaki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000097:blood; D023201:CD40 Ligand / Q000097:blood*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000097:blood*; D010392:Pemphigus / Q000097:blood; D012008:Recurrence