Aso Shotaro, Matsui Hiroki, Fushimi Kiyohide, Yasunaga Hideo
Department of Clinical Epidemiology and Health Economics, School of Public Health, The University of Tokyo, Tokyo, Japan.
Department of Health Policy and Informatics, Tokyo Medical and Dental University Graduate School of Medicine, Tokyo, Japan.
Sarcoidosis Vasc Diffuse Lung Dis. 2019;36(2):116-123. doi: 10.36141/svdld.v36i2.7181. Epub 2019 May 1.
Mortality of acute exacerbation of idiopathic pulmonary fibrosis is high, and it remains unknown whether cyclophosphamide is an effective treatment for this condition.
This study compared the effects of cyclophosphamide combined with systemic glucocorticoids with those of systemic glucocorticoids alone.
Using the Diagnosis Procedure Combination database in Japan, adult patients with idiopathic pulmonary fibrosis who had received high-dose methylprednisolone and mechanical ventilation at admission from July 1, 2010, to March 31, 2014, were identified. Instrumental variable analyses based on a hospital preference for cyclophosphamide were performed to compare in-hospital outcomes.
Eligible patients (n=1847) were divided into the methylprednisolone plus cyclophosphamide group (n=104) and the methylprednisolone alone group (n=1743). The results of an instrumental variable analysis detected no significant differences between the groups with respect to in-hospital mortality (odds ratio, 1.11; 95% confidence interval, 0.19-6.43), ventilator-free days (difference, 2.2; 95% confidence interval, -2.6 to 7.0).
In a Japanese inpatient database study analyzing outcomes from patients with acute exacerbation idiopathic pulmonary fibrosis receiving systemic glucocorticoids, the addition of cyclophosphamide was not associated with improved in-hospital mortality and ventilator-free days.
特发性肺纤维化急性加重的死亡率很高，环磷酰胺是否为该病的有效治疗方法仍不清楚。
本研究比较了环磷酰胺联合全身糖皮质激素与单纯全身糖皮质激素的效果。
利用日本的诊断程序组合数据库，确定了2010年7月1日至2014年3月31日期间入院时接受大剂量甲泼尼龙和机械通气的成年特发性肺纤维化患者。基于医院对环磷酰胺的偏好进行工具变量分析，以比较住院结局。
符合条件的患者（n = 1847）分为甲泼尼龙加环磷酰胺组（n = 104）和单纯甲泼尼龙组（n = 1743）。工具变量分析结果显示，两组在住院死亡率（优势比，1.11；95%置信区间，0.19 - 6.43）、无呼吸机天数（差异，2.2；95%置信区间，-2.6至7.0）方面无显著差异。
在一项对接受全身糖皮质激素治疗的特发性肺纤维化急性加重患者结局进行分析的日本住院患者数据库研究中，添加环磷酰胺与住院死亡率和无呼吸机天数的改善无关。
