Antineutrophil Cytoplasmic Antibodies and the Churg–Strauss Syndrome

1 November 2005 | Volume 143 Issue 9 | Pages 632-638

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.

*For a list of members of the French Vasculitis Study Group, see the Appendix.

Editors' Notes Context Patients with the Churg–Strauss syndrome, a rare small-sized vessel vasculitis, sometimes have antineutrophil cytoplasmic autoantibodies (ANCAs). Contribution In this cross-sectional study, 43 of 112 (38%) patients who recently had received a diagnosis of the Churg–Strauss syndrome were ANCA-positive. Compared with ANCA-negative patients, ANCA-positive patients more often had peripheral neuropathy (84% vs. 65%) and renal manifestations (35% vs. 4%). Also, ANCA-negative patients more often had fever (55% vs. 30%) and cardiac manifestations (49% vs. 12%). Implications Phenotypes of patients with ANCA-positive and ANCA-negative Churg–Strauss syndrome may differ. —The Editors

 

Author and Article Information

From Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Université Paris 5–René Descartes, Paris, France; Addenbrooks Hospital, Cambridge, United Kingdom; University Hospital Gasthuisberg, Leuven, Belgium; Hôpital Louis Pradel, Hospices Civils de Lyon, France; Hôpital Pontchaillou, Rennes, France; Centre Hospitalier, Le Mans, France; Hôpital Purpan, Toulouse, France; Hôpital Haut-Lévêque, Bordeaux, France; and Centre Hospitalier de la Côte de Nacre, Caen, France.

Grant Support: This work was funded by grants from the Ministère de la Recherche (UPRES 3409) and the Institut National de la Recherche Médicale (INSERM, GIS Maladies Rares). Patients were recruited with the help of the Hospices Civils de Lyon, which sponsored the therapeutic trials, the Assistance Publique–Hôpitaux de Paris, and the members of the French Vasculitis Study Group and the European Vasculitis Study Group (EUVAS).

Potential Financial Conflicts of Interest: None disclosed.

Requests for Single Reprints: Loïc Guillevin, MD, Department of Internal Medicine, Hôpital Cochin, Université Paris 5–René Descartes, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France; e-mail, loic.guillevin{at}cch.ap-hop-paris.fr.

Current Author Addresses: Dr. Sablé-Fourtassou: Department of Internal Medicine, Hôpital Avicenne, Université Paris 13, 125, rue de Stalingrad, 93009 Bobigny, France.

Drs. Cohen, Mahr, Pagnoux, Mouthon, and Guillevin: Department of Internal Medicine, Hôpital Cochin, Université Paris 5–René Descartes, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France.

Dr. Jayne: Department of Nephrology and Vasculitis, Renal Unit, Box 118, Addenbrookes Hospital, Cambridge CB2 2QQ, United Kingdom.

Dr. Blockmans: University Hospital Gasthuisberg, Dienst Interne Geneeskunde, Herestraat 49, B-3000 Leuven, Belgium.

Dr. Cordier: Department of Respiratory Medicine, Reference Center for Orphan Pulmonary Diseases, Hôpital Louis Pradel, Université Claude Bernard, 69677 Lyon (Bron), France.

Dr. Delaval: Department of Pneumologie, Hôpital Pontchaillou, rue Henri Le Guillou, 35033, Rennes, France.

Dr. Puechal: Department of Rheumatology, Centre Hospitalier Le Mans, 194, avenue Rubillard, 72037 Le Mans Cedex 9, France.

Dr. Lauque: Department of Internal Medicine and Pneumology, Hôpital Purpan, University of Toulouse, Place du Dr Baylac, 31059 Toulouse, Cedex, France.

Dr. Viallard: Department of Internal Medicine and Infectious Diseases, Hôpital du Haut-Lévêque, avenue de Magellan, 33604 Pessac, Cedex, France.

Dr. Zoulim: Department of Internal Mecicine, CHU Côte de Nacre, 14000 Caen, France.

Author Contributions: Conception and design: P. Cohen, D. Jayne, J.-F. Cordier, X. Puechal, L. Guillevin.

Analysis and interpretation of the data: R. Sablé-Fourtassou, P. Cohen, A. Mahr, C. Pagnoux, L. Mouthon, L. Guillevin.

Drafting of the article: R. Sablé-Fourtassou, A. Mahr, C. Pagnoux, L. Mouthon, L. Guillevin.

Critical revision of the article for important intellectual content: R. Sablé-Fourtassou, C. Pagnoux, L. Mouthon, D. Jayne, D. Blockmans, J.-F. Cordier, X. Puechal, L. Guillevin.

Final approval of the article: R. Sablé-Fourtassou, P. Cohen, A. Mahr, C. Pagnoux, L. Mouthon, D. Jayne, D. Blockmans, J.-F. Cordier, X. Puechal, D. Lauque, L. Guillevin.

Provision of study materials or patients: P. Cohen, C. Pagnoux, D. Jayne, D. Blockmans, J.-F. Cordier, P. Delaval, X. Puechal, D. Lauque, F. Viallard, A. Zoulim, L. Guillevin.

Statistical expertise: A. Mahr.

Obtaining of funding: L. Guillevin.

Administrative, technical, or logistic support: D. Jayne, L. Guillevin.

Collection and assembly of data: P. Cohen, C. Pagnoux, D. Jayne,