title: Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.
abstract: BACKGROUND
  Since testing for antineutrophil cytoplasmic antibodies (ANCA) became available for routine evaluation, no large homogeneous cohort of patients with the Churg-Strauss syndrome has been studied.
  
  
  OBJECTIVE
  To define the clinical and biological characteristics of newly diagnosed Churg-Strauss syndrome, according to the presence or absence of ANCA.
  
  
  METHODS
  Cross-sectional analysis of manifestations of participants who were enrolled in treatment trials between December 1995 and December 2002.
  
  
  METHODS
  Multicenter study in 63 clinical centers in France, Belgium, Latvia, and the United Kingdom, coordinated by the French Vasculitis Study Group.
  
  
  METHODS
  112 patients with Churg-Strauss syndrome that was recently diagnosed on the basis of current classifications.
  
  
  METHODS
  The authors compared principal demographic, clinical, and laboratory features according to ANCA status at diagnosis.
  
  
  RESULTS
  The authors detected ANCA in 43 (38%) patients. Positive ANCA status at diagnosis was associated with renal involvement, peripheral neuropathy, and biopsy-proven vasculitis, whereas negative ANCA status was associated with heart disease and fever.
  
  
  CONCLUSIONS
  The authors assessed ANCA by immunofluorescence, but they did not assess ANCA centrally or systematically retest if ANCA was undetected at diagnosis.
  
  
  CONCLUSIONS
  Phenotypically, ANCA-positive and ANCA-negative Churg-Strauss syndrome might differ. The association of ANCA positivity with clinical symptoms that indicate inflammation and necrosis of small vessels might characterize a predominantly vasculitic pattern of the Churg-Strauss syndrome.
authors.affiliation: Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5-René Descartes, Paris, France.
authors.forename: Régis
authors.initials: R
authors.lastname: Sablé-Fourtassou
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: David
authors.initials: D
authors.lastname: Jayne
authors.forename: Daniel
authors.initials: D
authors.lastname: Blockmans
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.forename: Philippe
authors.initials: P
authors.lastname: Delaval
authors.forename: Xavier
authors.initials: X
authors.lastname: Puechal
authors.forename: Dominique
authors.initials: D
authors.lastname: Lauque
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Viallard
authors.forename: Abdelkader
authors.initials: A
authors.lastname: Zoulim
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D015267:Churg-Strauss Syndrome / Q000097:blood* / Q000150:complications / Q000175:diagnosis; D003430:Cross-Sectional Studies; D005260:Female; D005334:Fever / Q000209:etiology; D006331:Heart Diseases / Q000209:etiology; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000209:etiology; D011379:Prognosis; D011446:Prospective Studies; D012720:Severity of Illness Index
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic