title: Churg-Strauss syndrome with poor-prognosis factors: A prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Arène
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Véronique
authors.initials: V
authors.lastname: Le Guern
authors.forename: Marie-Hélène
authors.initials: MH
authors.lastname: André
authors.forename: Martine
authors.initials: M
authors.lastname: Gayraud
authors.forename: David
authors.initials: D
authors.lastname: Jayne
authors.forename: Daniel
authors.initials: D
authors.lastname: Blöckmans
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000328:Adult; D000368:Aged; D018501:Antirheumatic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000401:mortality; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006801:Humans; D007275:Injections, Intravenous; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D020551:Pulse Therapy, Drug; D012008:Recurrence; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide