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

AbstractOBJECTIVE:
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.
AuthorsPascal 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, French Vasculitis Study Group
JournalArthritis and rheumatism (Arthritis Rheum) Vol. 57 Issue 4 Pg. 686-93 (May 15 2007) ISSN: 0004-3591 [Print] United States
PMID17471546 (Publication Type: Journal Article, Multicenter Study, Randomized Controlled Trial, Research Support, Non-U.S. Gov't)
Chemical References
  • Antirheumatic Agents
  • Glucocorticoids
  • Cyclophosphamide
Topics
  • Adult
  • Aged
  • Antirheumatic Agents (administration & dosage, adverse effects)
  • Churg-Strauss Syndrome (drug therapy, mortality)
  • Cyclophosphamide (administration & dosage, adverse effects)
  • Female
  • Glucocorticoids (administration & dosage, adverse effects)
  • Humans
  • Injections, Intravenous
  • Male
  • Middle Aged
  • Prognosis
  • Prospective Studies
  • Pulse Therapy, Drug
  • Recurrence
  • Survival Rate
  • Treatment Outcome

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