Sakamoto Susumu, Shimizu Hiroshige, Isshiki Takuma, Kurosaki Atsuko, Homma Sakae
Division of Respiratory Medicine, Toho University Omori Medical Center.
Department of Diagnostic Radiology, Fukujuji Hospital.
Sarcoidosis Vasc Diffuse Lung Dis. 2019;36(3):176-184. doi: 10.36141/svdld.v36i3.7750. Epub 2019 May 1.
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is often fatal. Although pharmacological treatments have been studied, outcomes remain poor. This study evaluated the effectiveness of pharmacological treatments for AE-IPF.
This retrospective study comprised 88 patients who received a diagnosis of AE-IPF and were admitted to our center during the period from January 2008 through April 2017. We reviewed the clinical features, treatments, and outcomes of the 88 patients. Cox proportional hazards regression analysis was used to identify variables that were significant predictors of 3-month death.
Data from 88 AE-IPF patients (age range, 56-81 years) were analyzed. In all patients, corticosteroid (CS) pulse therapy was performed an average of 1.7 times, and the initial CS maintenance dose was 1 mg/kg for 65 patients and 0.5 mg/kg for 23 patients. The combination treatments received were sivelestat in 83 patients (94%), recombinant human thrombomodulin (rhTM) in 45 patients (51%), pirfenidone in 41 patients (47%), and cyclosporine in 71 patients (81%). Univariate analysis showed that use of rhTM, and an initial CS maintenance dose of 0.5 mg/kg were associated with better 3-month survival. In multivariate analysis, both use of rhTM and an initial CS maintenance dose of 0.5 mg/kg were associated with better 3-month survival. Other treatments, including sivelestat, cyclosporine, pirfenidone, and polymyxin B-immobilized fiber column-direct hemoperfusion, were not associated with better 3-month survival.
Addition of rhTM to CS, and a low initial CS maintenance dose (0.5 mg/kg), were associated with better 3-month survival in patients with AE-IPF.
特发性肺纤维化急性加重（AE-IPF）通常是致命的。尽管已经对药物治疗进行了研究，但结果仍然很差。本研究评估了AE-IPF药物治疗的有效性。
这项回顾性研究纳入了2008年1月至2017年4月期间在本中心确诊为AE-IPF并入院的88例患者。我们回顾了这88例患者的临床特征、治疗方法和预后。采用Cox比例风险回归分析来确定预测3个月死亡率的显著变量。
分析了88例AE-IPF患者（年龄范围56 - 81岁）的数据。所有患者平均接受1.7次糖皮质激素（CS）脉冲治疗，65例患者初始CS维持剂量为1mg/kg，23例患者为0.5mg/kg。联合治疗药物中，83例患者（94%）使用了西维来司他，45例患者（51%）使用了重组人血栓调节蛋白（rhTM），41例患者（47%）使用了吡非尼酮，71例患者（81%）使用了环孢素。单因素分析显示，使用rhTM和初始CS维持剂量为0.5mg/kg与3个月更好的生存率相关。多因素分析表明，使用rhTM和初始CS维持剂量为0.5mg/kg均与3个月更好的生存率相关。其他治疗方法，包括西维来司他、环孢素、吡非尼酮和多黏菌素B固定纤维柱直接血液灌流，与3个月更好的生存率无关。
AE-IPF患者在CS治疗基础上加用rhTM以及较低的初始CS维持剂量（0.5mg/kg）与3个月更好的生存率相关。
