title: Monthly intravenous pulse cyclophosphamide therapy in Wegener's granulomatosis.
abstract: OBJECTIVE
  To study the long term effects of monthly intravenous cyclophosphamide therapy in Wegener's granulomatosis.
  
  
  METHODS
  Fourteen consecutive patients with active Wegener's granulomatos treated with a first-line combination of high-dose prednisone and monthly intravenous pulse cyclophosphamide were retrospectively studied.
  
  
  RESULTS
  One patient died from septicemia complicating severe leukopenia after the first pulse. At 8 months after instituting intravenous pulse cyclophosphamide therapy, failure was observed in 6 other patients. Between month 16 and 18, 2 other patients relapsed when the time between 2 pulses was lengthened. Five patients developed cyclophosphamide-related side-effects: infection (n = 2), amenorrhea (n = 1), alopecia (n = 2) and vomiting (n = 2). Except for one fatal infection, no major side-effect of intravenous cyclophosphamide therapy was observed. At the end of the study, all patients were off intravenous cyclophosphamide therapy with more than 6 months of followup. The 6 responders were in remission on low-dose prednisone or without treatment.
  
  
  CONCLUSIONS
  A combination of high-dose prednisone and intravenous cyclophosphamide may achieve long-term remission in 42% of patients with Wegener's granulomatosis. Responders to intravenous cyclophosphamide therapy had less extensive disease than non-responders.
authors.affiliation: Department of Internal Medicine, Groupe Hospitalier Pitié-Salpétrière, Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Lê Thi Huong
authors.forename: T
authors.initials: T
authors.lastname: Papo
authors.forename: J C
authors.initials: JC
authors.lastname: Piette
authors.forename: B
authors.initials: B
authors.lastname: Wechsler
authors.forename: O
authors.initials: O
authors.lastname: Blétry
authors.forename: G
authors.initials: G
authors.lastname: Lamas
authors.forename: A
authors.initials: A
authors.lastname: Baumelou
authors.forename: P
authors.initials: P
authors.lastname: Le Hoang
authors.forename: J L
authors.initials: JL
authors.lastname: Pennaforte
authors.forename: J C
authors.initials: JC
authors.lastname: Valcke
authors.forename: P
authors.initials: P
authors.lastname: Godeau
mesh_terms: D000293:Adolescent; D000328:Adult; D003520:Cyclophosphamide / Q000008:administration & dosage*; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage*; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage*; D012074:Remission Induction; D012189:Retrospective Studies
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone