title: An increase in circulating B cell-activating factor in childhood-onset ocular myasthenia gravis.
abstract: BACKGROUND
  Myasthenia gravis is a B cell-mediated autoimmune disorder. The pathophysiology of childhood-onset ocular myasthenia gravis remains unclear. We investigated serum B cell-activating factor levels and other immunological parameters in child patients with ocular myasthenia gravis.
  
  
  METHODS
  Blood samples were obtained from 9 children with ocular myasthenia gravis and 20 age-matched controls. We assayed serum concentrations of B cell-activating factor, anti-acetylcholine receptor antibody titers, 7 types of cytokines (interleukins-2, -4, -6, -10, and -17A; interferon-γ; tumor necrosis factor-α) as well as the percentages of peripheral blood CD4+, CD8+, and CD19+ cells.
  
  
  RESULTS
  Serum B cell-activating factor levels were significantly higher before immunosuppressive therapy in patients with childhood-onset ocular myasthenia gravis than in controls and decreased after immunosuppressive therapy. A significant positive correlation was observed between serum B cell-activating factor levels and anti-acetylcholine receptor antibody titers in patients with myasthenia gravis. Serum B cell-activating factor concentrations did not correlate with the percentages of CD4+, CD8+, and CD19+ cells or the CD4+/CD8+ ratio. No significant differences were observed in the levels of the 7 different types of cytokines examined, including interleukin-17A, between preimmunosuppressive therapy myasthenia gravis patients and controls.
  
  
  CONCLUSIONS
  Circulating B cell-activating factor may play a key role in the pathophysiology of childhood-onset ocular myasthenia gravis.
authors: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors: Mitsuo
authors: M
authors: Motobayashi
authors: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan. Electronic address: inabay@shinshu-u.ac.jp.
authors: Yuji
authors: Y
authors: Inaba
authors: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors: Takafumi
authors: T
authors: Nishimura
authors: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors: Norimoto
authors: N
authors: Kobayashi
authors: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors: Yozo
authors: Y
authors: Nakazawa
authors: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors: Kenichi
authors: K
authors: Koike
mesh_terms: D000293:Adolescent; D017668:Age of Onset; D001323:Autoantibodies / Q000097:blood; D053264:B-Cell Activating Factor / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D016207:Cytokines / Q000097:blood; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D007223:Infant; D008297:Male; D009157:Myasthenia Gravis / Q000097:blood* / Q000188:drug therapy; D011950:Receptors, Cholinergic / Q000276:immunology; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D053264:B-Cell Activating Factor; D016207:Cytokines; D011950:Receptors, Cholinergic
keywords: B-cell activating factor; IL-17; Th17; childhood-onset ocular myasthenia gravis