Monthly intravenous pulse cyclophosphamide therapy in Wegener's granulomatosis.
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.
Lê Thi Huong
D
D
Department of Internal Medicine, Groupe Hospitalier Pitié-Salpétrière, Paris, France.
Papo
T
T
Piette
J C
JC
Wechsler
B
B
Blétry
O
O
Lamas
G
G
Baumelou
A
A
Le Hoang
P
P
Pennaforte
J L
JL
Valcke
J C
JC
Godeau
P
P
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
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone