Várai G, Earle L, Jimenez S A, Steiner R M, Varga J
Department of Radiology, Jefferson Medical College, Philadelphia, Pennsylvania, USA.
J Rheumatol. 1998 Jul;25(7):1325-9.
Pulmonary fibrosis, a frequent manifestation of systemic sclerosis (SSc), is considered incurable. Our aim was to assess the effect of therapy with intravenous (i.v.) cyclophosphamide on the course of pulmonary fibrosis in patients with SSc.
Five patients with SSc and clinical, laboratory, or radiographic findings of interstitial lung disease were treated with cyclophosphamide (1 g) administered i.v. monthly for 48 weeks. The dyspnea score, pulmonary function tests, arterial blood oxygen content, radiologic abnormalities, and bronchoalveolar lavage (BAL) fluid cellularity were determined before and after therapy.
The dyspnea score decreased by 42% after 48 weeks of therapy. Forced vital capacity (FVC, percentage of predicted) increased by 7%, and carbon monoxide diffusing capacity (DLCO) decreased by 12%, but these changes were not statistically significant. Arterial blood oxygenation remained unchanged. At baseline, high resolution computed tomography of the lungs showed honeycombing, reticulonodular, or ground glass patterns in each patient examined. These radiologic abnormalities did not improve during treatment. A marked decrease in BAL fluid cell number, but not in the percentage of neutrophils, was observed after therapy. Nausea and leukopenia were frequent but mild side effects. One patient developed hemorrhagic cystitis.
The results suggest that intermittent treatment with i.v. cyclophosphamide reduces the severity of dyspnea, but fails to improve FVC or DLCO, or cause resolution of radiologic abnormalities, in patients with SSc.
肺纤维化是系统性硬化症（SSc）的常见表现，被认为无法治愈。我们的目的是评估静脉注射环磷酰胺治疗对SSc患者肺纤维化病程的影响。
5例有SSc且伴有间质性肺病临床、实验室或影像学表现的患者接受环磷酰胺（1g）静脉注射治疗，每月1次，共48周。在治疗前后测定呼吸困难评分、肺功能测试、动脉血氧含量、放射学异常以及支气管肺泡灌洗（BAL）液细胞成分。
治疗48周后呼吸困难评分下降了42%。用力肺活量（FVC，预测值百分比）增加了7%，一氧化碳弥散量（DLCO）下降了12%，但这些变化无统计学意义。动脉血氧合情况保持不变。基线时，肺部高分辨率计算机断层扫描显示每位受检患者均有蜂窝状、网状结节状或磨玻璃样改变。治疗期间这些放射学异常未改善。治疗后观察到BAL液细胞数量显著减少，但中性粒细胞百分比未减少。恶心和白细胞减少是常见但轻微的副作用。1例患者发生出血性膀胱炎。
结果表明，对于SSc患者，静脉注射环磷酰胺间歇治疗可减轻呼吸困难的严重程度，但未能改善FVC或DLCO，也未使放射学异常消失。
