环磷酰胺冲击治疗对间质性肺病有效

作者：Schupp JC，翻译：金银姬，校对：武东

摘要：背景：间质性肺病（ILD）是一组具有进行性肺功能下降的疾病。我们观察到系统性硬化症患者接受间歇性环磷酰胺静脉注射后肺功能维持稳定，但目前关于间质性肺病患者，其相关数据欠缺。

目的：回顾性评估环磷酰胺冲击治疗在间质性肺病的有效性。

方法：我们回顾性分析了2002年至2012年接受环磷酰胺静脉注射的所有患者。18个月随访期间记录肺功能检查、生存状态及支气管肺泡灌洗液细胞学检查等数据。

结果：本研究纳入了26名特发性肺纤维化患者、6名淋巴细胞性间质性肺炎（LIP）、8名特发性非特异性间质性肺炎（NSIP）、7名类风湿关节炎相关性间质性肺炎及7名pANCA阳性相关性间质性肺病。结果显示，淋巴细胞性间质性肺炎及特发性非特异性间质性肺炎患者预后最好，pANCA阳性的相关性间质性肺病患者预后最差。整个队列中，我们发现与治疗后一年相比，治疗前一年的肺总量明显下降。

结论：这项回顾性分析显示，接受环磷酰胺治疗的大部分纤维化性相关性间质性肺病患者肺功能稳定，迫切需要进一步明确诊断的前瞻性研究。

附原文：Abstract BACKGROUND: Interstitial lung diseases (ILDs) are a group of disorders characterised by progressive lung function decline. Stabilisation of lungfunction under intermittent i.v. cyclophosphamide was shown in patients suffering from systemic sclerosis, yet data in ILD patients are scarce. OBJECTIVES: To retrospectively evaluate the usefulness of cyclophosphamide pulse therapy in ILD. METHODS: We retrospectively analysed all patients who received i.v. cyclophosphamide in our centre from 2002 to 2012. Lung function, survival status, and bronchoalveolar lavage cytology were recorded during a follow-up period of 18 months.RESULTS:Twenty-six patients with idiopathic pulmonary fibrosis, 6 with lymphocytic interstitial pneumonia (LIP), 8 with idiopathic non-specificinterstitial pneumonia (NSIP), 7 with rheumatoid arthritis-associated ILD, and 7 with perinuclear anti-neutrophil cytoplasmic antibody-positive ILD (pANCA+ ILD) were included. Patients with LIP and NSIP had the best survival outcome, those with pANCA+ ILD the worst. In the total cohort, we found a significantly higher total lung capacity decline in the year before treatment compared to the year after treatment.CONCLUSIONS:This retrospective analysis of cyclophosphamide treatment shows a stabilisation of lung function in most patients with fibrotic ILDs, yet prospective studies in clearly defined diagnoses are urgently needed.

引自：Schupp JC, K?hler T, Müller-Quernheim J. Usefulness of Cyclophosphamide Pulse Therapy in Interstitial Lung Diseases.Respiration. 2016 Apr 16. [Epub ahead of print]