Churg-Strauss syndrome with poor-prognosis factors: A prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients.
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.
Cohen
Pascal
P
Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Université René Descartes Paris, Recherche Clinique et Thérapeutique, Paris, France.
Pagnoux
Christian
C
Mahr
Alfred
A
Arène
Jean-Pierre
JP
Mouthon
Luc
L
Le Guern
Véronique
V
André
Marie-Hélène
MH
Gayraud
Martine
M
Jayne
David
D
Blöckmans
Daniel
D
Cordier
Jean-François
JF
Guillevin
Loïc
L
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
D018501:Antirheumatic Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide