Churg‐Strauss syndrome with poor‐prognosis factors: A prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty‐eight patients |
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| Authors: | Pascal Cohen Christian Pagnoux Alfred Mahr Jean‐Pierre Arène Luc Mouthon Véronique Le Guern Marie‐Hélène André Martine Gayraud David Jayne Daniel Blöckmans Jean‐franÇois Cordier Loïc Guillevin The French Vasculitis Study Group |
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| Institution: | 1. H?pital Cochin, Assistance Publique H?pitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France;2. Addenbrookes Hospital, Cambridge, UK;3. Dr. Jayne has received consulting fees and/or honoraria (less than $10,000 each) from Roche, Novartis, and Genzyme.;4. University Hospital Gasthuisberg, Leuven, Belgium;5. H?pital Louis Pradel, Lyon, France |
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| 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. |
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| Keywords: | Churg‐Strauss Vasculitis Therapy Glucocorticoids Cyclophosphamide |
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