Lê Thi Huong D, Papo T, Piette J C, Wechsler B, Blétry O, Lamas G, Baumelou A, Le Hoang P, Pennaforte J L, Valcke J C, Godeau P
Department of Internal Medicine, Groupe Hospitalier Pitié-Salpétrière, Paris, France.
Clin Exp Rheumatol. 1996 Jan-Feb;14(1):9-16.
To study the long term effects of monthly intravenous cyclophosphamide therapy in Wegener's granulomatosis.
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.
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.
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.
研究每月静脉注射环磷酰胺治疗韦格纳肉芽肿的长期效果。
回顾性研究了14例连续接受一线大剂量泼尼松和每月静脉脉冲环磷酰胺联合治疗的活动性韦格纳肉芽肿患者。
1例患者在首次脉冲治疗后死于并发严重白细胞减少的败血症。在开始静脉脉冲环磷酰胺治疗8个月后，另外6例患者出现治疗失败。在第16至18个月期间，当两次脉冲治疗的间隔时间延长时，又有2例患者复发。5例患者出现了与环磷酰胺相关的副作用：感染（2例）、闭经（1例）、脱发（2例）和呕吐（2例）。除1例致命感染外，未观察到静脉注射环磷酰胺治疗的重大副作用。在研究结束时，所有患者均停止静脉注射环磷酰胺治疗，随访时间超过6个月。6例有反应的患者在低剂量泼尼松治疗或未治疗的情况下处于缓解状态。
大剂量泼尼松和静脉注射环磷酰胺联合治疗可使42%的韦格纳肉芽肿患者获得长期缓解。对静脉注射环磷酰胺治疗有反应的患者病变范围比无反应者小。
