Churg-Strauss syndrome with poor-prognosis factors: A prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients

Authors

  • Pascal Cohen,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Christian Pagnoux,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Alfred Mahr,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Jean-Pierre Arène,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Luc Mouthon,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Véronique Le Guern,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Marie-Hélène André,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • Martine Gayraud,

    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
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  • David Jayne,

    1. Addenbrookes Hospital, Cambridge, UK
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    • Dr. Jayne has received consulting fees and/or honoraria (less than $10,000 each) from Roche, Novartis, and Genzyme.

  • Daniel Blöckmans,

    1. University Hospital Gasthuisberg, Leuven, Belgium
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  • Jean-franÇois Cordier,

    1. Hôpital Louis Pradel, Lyon, France
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  • Loïc Guillevin,

    Corresponding author
    1. Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France
    • Department of Internal Medicine, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris 5, UPRES 3409 Recherche Clinique et Thérapeutique, 27 rue du Faubourg Saint Jacques, 75679 Paris Cedex 14, Paris, France
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  • The French Vasculitis Study Group


Abstract

Objective

To compare long and short durations of adjunctive cyclophosphamide for the treatment of severe Churg-Strauss syndrome (CSS).

Methods

In this prospective multicenter therapeutic trial, 48 patients with CSS with at least 1 poor-prognosis factor at baseline were treated with glucocorticoids and either 12 or 6 intravenous cyclophosphamide pulses.

Results

At 8 years, complete remission rates and severe side effects of therapy were comparable for both groups. The overall difference in relapses was not significant between the 12-pulse and the 6-pulse regimens (P = 0.07), but when considering only the number of mild relapses this difference became statistically significant (P < 0.02). Although the total number of inclusions was not reached, the study was stopped prematurely in response to the superiority of the 12-pulse regimen.

Conclusion

We concluded that 12 cyclophosphamide pulses were better able to control severe CSS than a 6-pulse regimen. The optimal duration of therapy remains to be determined.

Ancillary