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.

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.

• Authors: Régis Sablé-Fourtassou, Pascal Cohen, Alfred Mahr, Christian Pagnoux, Luc Mouthon, David Jayne, Daniel Blockmans, Jean-François Cordier, Philippe Delaval, Xavier Puechal, Dominique Lauque, Jean-François Viallard, Abdelkader Zoulim, Loïc Guillevin, French Vasculitis Study Group
• Journal: Annals of internal medicine (Ann Intern Med) Vol. 143 Issue 9 Pg. 632-8 (Nov 01 2005) ISSN: 1539-3704 [Electronic] United States
• PMID: 16263885 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)

Chemical References

• Antibodies, Antineutrophil Cytoplasmic

Topics

• Adolescent
• Adult
• Aged
• Antibodies, Antineutrophil Cytoplasmic (immunology)
• Churg-Strauss Syndrome (blood, complications, 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