Servicio de Enfermedades Autoinmunes Sistémicas, Institut Clínic de Medicina i Dermatologia, Hospital Clinic, Barcelona, España.
Arch Bronconeumol. 2011 May;47(5):239-45. doi: 10.1016/j.arbres.2011.01.010. Epub 2011 Mar 31.
Cyclophosphamide (CYC) stabilizes the parameters of lung function tests (LFT) of patients with (SSc) and interstitial lung disease (ILD) treated for 12 months. There is little information about long-term treatment (24 months). The aim of this study is to analyze the effect of intravenous CYC in LFT parameters in patients with SSc and ILD treated for 24 months.
Retrospective study of 37 patients with ILD associated with scleroderma treated with intravenous CYC for 24 months and regularly assessed by LFT (at baseline, 6, 12 and 24 months) including forced vital capacity (FVC) and transfer capacity of carbon monoxide (DL(CO)). To evaluate response to treatment the recommendations of the ATS and SEPAR were considered.
The difference between FVC and DL(CO) values performed at baseline and those performed at 6, 12, and 24 months were less than 10%, which meant that CYC stabilized LFT. There were no differences in LFT when patients treated for 6 months were evaluated according to the type of skin involvement of the SSc (diffuse or limited) and the duration of the ILD. Although patients with severe restriction (FVC<70%) showed more improvement, it was less than 10% in all cases.
In this series of patients with ILD associated with SSc intravenous CYC was effective in stabilizing LFT in long-term treatment.
环磷酰胺（CYC）可稳定接受治疗 12 个月的系统性硬皮病（SSc）和间质性肺病（ILD）患者的肺功能测试（LFT）参数。关于长期治疗（24 个月）的信息较少。本研究旨在分析静脉注射 CYC 对接受 24 个月治疗的 SSc 和 ILD 患者的 LFT 参数的影响。
对 37 例接受静脉注射 CYC 治疗 24 个月的伴有硬皮病的ILD 患者进行回顾性研究，并定期通过 LFT（基线、6、12 和 24 个月）进行评估，包括用力肺活量（FVC）和一氧化碳转移能力（DL（CO））。为了评估治疗反应，考虑了 ATS 和 SEPAR 的建议。
基线时 FVC 和 DL（CO）值与 6、12 和 24 个月时的差值小于 10%，这意味着 CYC 稳定了 LFT。根据 SSc 的皮肤受累类型（弥漫性或局限性）和ILD 的持续时间评估接受 6 个月治疗的患者时，LFT 没有差异。尽管严重受限（FVC<70%）的患者显示出更多的改善，但所有患者的改善均小于 10%。
在本系列 SSc 相关ILD 患者中，静脉注射 CYC 在长期治疗中有效稳定了 LFT。
