title: Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients.
abstract: OBJECTIVE
  Because the optimal cyclophosphamide (CY) treatment duration for severe polyarteritis nodosa (PAN) without virus infection and microscopic polyangiitis (MPA) has not been established, we conducted a trial to compare the effectiveness of 6 versus 12 CY pulses given in combination with corticosteroids (CS).
  
  
  METHODS
  Sixty-five (18 PAN, 47 MPA) previously untreated patients were randomized to receive 12 (n = 34) or 6 (n = 31) CY pulses combined with CS. PAN and MPA were histologically proven or met ACR criteria. All patients presented >or=1 factor of severity according to the five factor score (FFS). CY pulses were administered every 2 weeks for 1 month, then every 4 weeks. The end point of the study was the number of events (relapses and/or deaths) occurring in each group, analyzed according to an intention-to-treat strategy. The outcome was evaluated by Cox proportional hazards analysis.
  
  
  RESULTS
  The baseline characteristics were similar for both groups. The mean (+/- SD) followup was 32 +/- 21 months. Survival analysis showed a significantly lower relapse probability (P = 0.02; hazards ratio [HR] = 0.34) and higher event-free survival (P = 0.02, HR = 0.44) for the 12 CY-pulse group while the mortality rates were not significantly different (P = 0.47).
  
  
  CONCLUSIONS
  These results suggest that 6 CY pulses are less effective than 12 CY pulses to treat severe PAN and MPA, particularly with respect to the risk of relapses.
authors.affiliation: Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, Université Paris-Nord, Bobigny, France. loic.guillevin@avc.ap-hop-paris.fr
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
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: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.forename: Edouard
authors.initials: E
authors.lastname: Pertuiset
authors.forename: Brigitte
authors.initials: B
authors.lastname: Gilson
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.forename: Patricia
authors.initials: P
authors.lastname: Lanoux
authors.forename: Alain
authors.initials: A
authors.lastname: Bruet
authors.forename: Marc
authors.initials: M
authors.lastname: Ruivard
authors.forename: Philippe
authors.initials: P
authors.lastname: Vanhille
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Cordier
mesh_terms: D000328:Adult; D000368:Aged; D018501:Antirheumatic Agents / Q000008:administration & dosage* / Q000009:adverse effects; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage* / Q000009:adverse effects; D006801:Humans; D008297:Male; D008833:Microcirculation; D008875:Middle Aged; D010488:Polyarteritis Nodosa / Q000188:drug therapy* / Q000401:mortality; D011379:Prognosis; D011446:Prospective Studies; D020551:Pulse Therapy, Drug; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D005938:Glucocorticoids; D003520:Cyclophosphamide