MONTHLY INTRAVENOUS PULSE CYCLOPHOSPHAMIDE THERAPY IN WEGENERS GRANULOMATOSIS

Objective. To study the long term effects of monthly intravenous cyclo phosphamide therapy in Wegener's granulomatosis. Methods. Fourteen con secutive patients with active Wegener's granulomatos treated with a fi rst-line combination of high-dose prednisone and monthly intravenous p ulse cyclophosphamide were retrospectively studied. Results. One patie nt died from septicemia complicating severe leukopenia after the first pulse. At 8 months after instituting intravenous pulse cyclophosphami de 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-effec ts: infection (n = 2), amenorrhea (n = 1), alopecia (n = 2) and vomiti ng (n = 2). Except for one fatal infection, no major side-effect of in travenous cyclophosphamide therapy was observed At the end ofthe study , all patients were off intravenous cyclophosphamide therapy with move than 6 months of followup. The 6 responders were in remission on low- dose prednisone or without treatment. Conclusion. A combination of hig h-dose prednisone and intravenous cyclophosphamide may achieve long-te rm remission in 42% of patients with Wegener's granulomatosis. Respond ers to intravenous cyclophosphamide therapy had less extensive disease than non-responders.