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Antineutrophil Cytoplasmic Antibodies and the Churg–Strauss Syndrome

Régis Sablé-Fourtassou, MD; Pascal Cohen, MD; Alfred Mahr, MD; Christian Pagnoux, MD; Luc Mouthon, MD, PhD; David Jayne, MD; Daniel Blockmans, MD, PhD; Jean-François Cordier, MD; Philippe Delaval, MD; Xavier Puechal, MD, PhD; Dominique Lauque, MD; Jean-François Viallard, MD, PhD; Abdelkader Zoulim, MD; Loïc Guillevin, MD, the French Vasculitis Study Group*
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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@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,

Ann Intern Med. 2005;143(9):632-638. doi:10.7326/0003-4819-143-9-200511010-00006
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We believe that our study is the first to report the results of a prospective multicenter study that compared patients with the Churg–Strauss syndrome as a function of their ANCA status at diagnosis. The strengths of our study include the homogeneous management (that is, we included patients within a limited period of time, investigated them in the same manner, and recorded their data prospectively) and the large number of centers and medical specialties that participated in patient recruitment, which is representative of the Churg–Strauss syndrome in its different clinical aspects.

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Study flow chart.

The flow chart shows clinical status of patients with the Churg–Strauss syndrome as a function of their antineutrophil cytoplasmic antibody (ANCA) positivity or negativity. One hundred one patients (37 ANCA-positive, 64 ANCA-negative) were alive at last update.

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