Reductions in pulmonary function detected in patients with lymphangioleiomyomatosis: An analysis of the Japanese National Research Project on Intractable Diseases database
Reductions in pulmonary function detected in patients with lymphangioleiomyomatosis: An analysis of the Japanese National Research Project on Intractable Diseases database
复制标题
DOI：
10.1016/j.resinv.2015.11.003
复制
发表时间：
2016-05-01
影响因子：
3.1
通讯作者：
Mishima, Michiaki
中科院分区：
文献类型：
--
作者：
Hayashida, Mie;Yasuo, Masanori;Mishima, Michiaki
背景：在淋巴管平滑肌瘤病（LAM）中，预测肺部疾病进展对治疗计划至关重要。然而，以前没有日本的研究试图预测LAM患者发生的肺功能下降。方法：在排除接受过以下检查的患者后，分析了2009年10月至2014年3月期间接受过> 3次肺量测定检查且数据已在日本国家难治性疾病研究项目数据库中登记的89例LAM患者的数据：（1）肺移植;（2）mTOR抑制剂治疗;或（3）胸腔引流、胸膜固定术、手术或胸导管结扎。结果：在整个研究人群中，第一秒用力呼气量（FEV 1）斜率的中位数（四分位数）为46.7（-95.2; -15.0）mL/年。研究期间，发现保守治疗气胸的发作与FEV 1（%预测值）的快速降低相关。研究期间妊娠与FEV 1（%预测值）降低相关。当患者被分为显示初始FEV 1（%预测值）值>70%（A组）和
Background: In lymphangioleiomyomatosis (LAM), predicting lung disease progression is essential for treatment planning. However, no previous Japanese studies have attempted to predict the reductions in pulmonary function that occur in LAM patients.Methods: The data for 89 LAM patients who had undergone > 3 spirometry tests and whose data had been registered in the Japanese National Research Project on Intractable Diseases database between October 2009 and March 2014 were analyzed after excluding patients who had undergone (1) a lung transplant; (2) mTOR inhibitor treatment; or (3) thoracic drainage, pleurodesis, surgery, or thoracic duct ligation during the study period. The rates of change (slope) in pulmonary parameters were calculated, and their associations with clinical background factors were investigated.Results: Among the whole study population, the median (quartiles) slope of forced expiratory volume in one second (FEV1) was 46.7 (-95.2; -15.0) mL per year. Episodes of conservatively treated pneumothorax during the study period were found to be associated with rapid reductions in FEV1 (% predicted). Pregnancy during the study period was associated with a reduction in FEV1 (% predicted). When the patients were divided into those who exhibited initial FEV1 (% predicted) values of >70% (Group A) and