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研究33例有活动后呼吸困难症状的特发性肺纤维化患者(病情恶化,或有应用皮质激素的禁忌证),以确定静注环磷酰胺的安全性和疗效。患者均接受两周1次静注环磷酰胺治疗,开始剂量为500mg.若白细胞计数在3.0×10~9/L 以上,则剂量逐渐增加,最大剂量为1000～1500mg;在患者能耐受的情况下,激素治疗逐渐减量。最少治疗6个月或直至死亡。结果33例患者18个月生存率超过50%。存活6个月的患者,肺活量显著增加,从1.6±0.61L 升至1.8±0.52L(P<0.01),并在治疗的18个月期间一直维持。与此同时,在12个月内平均强的松剂量显著
33 patients with idiopathic pulmonary fibrosis who had dyspnea after their activity (exacerbations or contraindications to corticosteroids) were studied to determine the safety and efficacy of intravenous cyclophosphamide. Patients were treated with intravenous cyclophosphamide once a week for 2 weeks and the initial dose was 500 mg.If the white blood cell count was above 3.0 × 10 9 / L, the dosage gradually increased to a maximum of 1000 to 1500 mg. In the case of hormone therapy tapering. At least 6 months treatment or until death. Results The 33-month survival rate was over 50% at 18 months. Patients who survived for 6 months had a significant increase in vital capacity, from 1.6 ± 0.61 L to 1.8 ± 0.52 L (P <0.01), and were maintained during the 18 months of treatment. In the meantime, the average prednisone dose was significant within 12 months