title: Antineutrophil cytoplasmic autoantibodies and myeloperoxidase autoantibodies in clinical expression of Churg-Strauss syndrome.
abstract: BACKGROUND
  The clinical significance of antineutrophil cytoplasmic antibodies (ANCAs) in the phenotypic expression of Churg-Strauss syndrome (CSS) is uncertain.
  
  
  OBJECTIVE
  We sought to investigate the relationship between ANCA status and the clinical expression of CSS in a case series derived from the US Food and Drug Administration's adverse events database.
  
  
  METHODS
  All cases of CSS reported to the US Food and Drug Administration from 1997 to April 2003 were reviewed. Information about basic demographics, suspect medication use, clinical manifestations, histologic findings, ANCA staining patterns, and the presence of antibodies to myeloperoxidase (anti-MPO) or proteinase 3 (anti-PR3) was recorded when available.
  
  
  RESULTS
  There were 93 case reports of CSS with sufficient documentation, including ANCA status. There were 38 (40.9%) of 93 cases with positive ANCA results, of which 15 cases reported a positive ELISA, all of which were positive for anti-MPO. ANCA negativity was associated with an increased proportion of cardiac involvement (risk difference [RD], 38.2%; 95% CI, 25.3% to 51.0%), gastrointestinal involvement (RD, 25.5%; 95% CI, 13.9% to 37.0%), pulmonary infiltrates (odds ratio, 4.9; 95% CI, 1.5-16.2), and the outcome of a life-threatening event or death (RD, 30.9%; 95% CI, 18.7% to 43.1%) when compared with anti-MPO-positive cases. ANCA negativity was associated with a decreased proportion of peripheral neuropathy (odds ratio, 0.3; 95% CI, 0.07-0.9).
  
  
  CONCLUSIONS
  These findings support the hypothesis that the presence or absence of autoantibodies influences the clinical expression and severity of CSS.
authors.affiliation: Medical Research Institute of New Zealand, Wellington, New Zealand.
authors.forename: Bridget
authors.initials: B
authors.lastname: Healy
authors.forename: Susan
authors.initials: S
authors.lastname: Bibby
authors.forename: Richard
authors.initials: R
authors.lastname: Steele
authors.forename: Mark
authors.initials: M
authors.lastname: Weatherall
authors.forename: Harold
authors.initials: H
authors.lastname: Nelson
authors.forename: Richard
authors.initials: R
authors.lastname: Beasley
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology*; D015267:Churg-Strauss Syndrome / Q000201:enzymology / Q000276:immunology* / Q000473:pathology*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D006801:Humans; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D009195:Peroxidase; D053146:Myeloblastin