Silver R M, Warrick J H, Kinsella M B, Staudt L S, Baumann M H, Strange C
Department of Medicine, Medical University of South Carolina, Charleston 29425.
J Rheumatol. 1993 May;20(5):838-44.
Fourteen patients with systemic sclerosis (SSc, scleroderma) and interstitial lung disease were treated with oral cyclophosphamide (1-2 mg/kg/day) and low dose prednisone (< 10 mg/day). There was a significant improvement in FVC after 6 months compared to entry values (2.21 +/- 0.19 l vs. 2.03 +/- 0.15 l, p < 0.02). Improvement was maintained at 12 months (2.27 +/- 0.27 l, p < 0.05) and 18-24 months (2.60 +/- 0.28 l, p < 0.001). In 12 cases followed for 18-24 months, FVC was stable or improved. No significant improvement or decline was noted for the DLCO. Side effects included cytopenia (2), infection (1), and hemorrhagic cystitis (2), and one possible related malignancy. A controlled prospective trial of cyclophosphamide is warranted in patients with SSc and active interstitial lung disease.
14例系统性硬化症（SSc，硬皮病）合并间质性肺病患者接受口服环磷酰胺（1-2mg/kg/天）及小剂量泼尼松（<10mg/天）治疗。与入组时相比，6个月后用力肺活量（FVC）有显著改善（2.21±0.19L对2.03±0.15L，p<0.02）。12个月时改善仍持续（2.27±0.27L，p<0.05），18-24个月时（2.60±0.28L，p<0.001）亦是如此。在12例随访18-24个月的患者中，FVC稳定或改善。一氧化碳弥散量（DLCO）未观察到显著改善或下降。副作用包括血细胞减少（2例）、感染（1例）、出血性膀胱炎（2例）以及1例可能与之相关的恶性肿瘤。对于患有SSc和活动性间质性肺病的患者，有必要进行环磷酰胺的对照前瞻性试验。
