Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.
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.
Sablé-Fourtassou
Régis
R
Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris 5-René Descartes, Paris, France.
Cohen
Pascal
P
Mahr
Alfred
A
Pagnoux
Christian
C
Mouthon
Luc
L
Jayne
David
D
Blockmans
Daniel
D
Cordier
Jean-François
JF
Delaval
Philippe
P
Puechal
Xavier
X
Lauque
Dominique
D
Viallard
Jean-François
JF
Zoulim
Abdelkader
A
Guillevin
Loïc
L
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
D019268:Antibodies, Antineutrophil Cytoplasmic