Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome

Ann Intern Med. 2005 Nov 1;143(9):632-8. doi: 10.7326/0003-4819-143-9-200511010-00006.


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.

Design: Cross-sectional analysis of manifestations of participants who were enrolled in treatment trials between December 1995 and December 2002.

Setting: Multicenter study in 63 clinical centers in France, Belgium, Latvia, and the United Kingdom, coordinated by the French Vasculitis Study Group.

Participants: 112 patients with Churg-Strauss syndrome that was recently diagnosed on the basis of current classifications.

Measurements: 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.

Limitations: 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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Churg-Strauss Syndrome / blood*
  • Churg-Strauss Syndrome / complications
  • Churg-Strauss Syndrome / diagnosis
  • Cross-Sectional Studies
  • Female
  • Fever / etiology
  • Heart Diseases / etiology
  • Humans
  • Kidney Diseases / etiology
  • Male
  • Middle Aged
  • Peripheral Nervous System Diseases / etiology
  • Prognosis
  • Prospective Studies
  • Severity of Illness Index


  • Antibodies, Antineutrophil Cytoplasmic