WEGENERS GRANULOMATOSIS - LONG-TERM FOLLOW-UP OF PATIENTS TREATED WITH PULSE CYCLOPHOSPHAMIDE

Treatment with daily oral cyclophosphamide (CY) has improved survival in Wegener's granulomatosis (WG), but is associated with severe and po tentially lethal adverse effects. Less toxic treatment regimens, such as pulse CY, have been used, but the effect has been questioned. We ha ve treated 11 patients with WG with pulse CY (15 mg/kg initially every second week, gradually increasing the pulse interval). After 4.5 yr f ollow-up and a total of 501 pulses of CY, one patient died and eight p atients (73%) were in complete remission. Remission was induced in 91% of the patients after a median period of 3.5 months and relapses were seen in 60%. With the same treatment protocol, a new complete remissi on was induced in 75% of those relapsing. Except for one patient who d ied, no patient developed end-stage renal failure. Haemorrhagic cystit is was not observed and no malignancies recorded. Severe infections we re seen in 36%, but none caused by Pneumocystis carinii. Nausea was th e most frequent side-effect, seen in 63% of the patients. We conclude that treatment with pulse CY every second week is safe and effective i n inducing remission and treating relapses in WG. The relapse rate see ms to be higher than with low-dose oral CY, but the cumulative dose of CY is less.