OBJECTIVE The aim of this study is to describe and clarify the factors affecting the prognosis of Japanese patients with amyotrophic lateral sclerosis (ALS) undergoing tracheostomy invasive ventilation (TIV) therapy. METHODS We conducted a prospective longitudinal observational case-control study using a multicentre registry. ALS patients who started TIV therapy after registration (TIV group) and those who did not receive TIV (non-TIV group) were included. We compared the survival time between the TIV group and the non-TIV group using a propensity score matching analysis and evaluated the prognostic factors in the TIV group. RESULTS From February 2006 to January 2018, 190 patients in the TIV group and 1093 patients in the non-TIV group were included in this study. The mean age of disease onset and usage rate of gastrostomy and non-invasive ventilation therapy differed between the groups. In the propensity score matching analysis using known prognostic factors, the median overall survival time of the TIV group was significantly greater than that of the non-TIV group (11.33 years vs 4.61 years; p<0.001). Analysis using the Cox proportional hazard model suggested that older age of onset and respiratory onset was an independent factor for poor prognosis after starting TIV therapy. CONCLUSION We showed that there was a significant difference of approximately 7 years in life expectancy between Japanese ALS patients who did and did not receive TIV therapy.

中文翻译：

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日本进行气管切开术有创通气治疗的肌萎缩性侧索硬化患者的预后。

目的本研究的目的是描述和阐明影响日本气管萎缩侧索硬化症（ALS）接受气管切开术有创通气（TIV）治疗的日本患者预后的因素。方法我们使用多中心注册表进行了一项前瞻性纵向观察病例对照研究。登记后开始TIV治疗的ALS患者（TIV组）和未接受TIV的ALS患者（非TIV组）包括在内。我们使用倾向评分匹配分析比较了TIV组和非TIV组的生存时间，并评估了TIV组的预后因素。结果从2006年2月到2018年1月，TIV组的190例患者和非TIV组的1093例患者被纳入本研究。两组患者的平均发病年龄，胃造口术和无创通气治疗的使用率不同。在使用已知预后因素进行的倾向评分匹配分析中，TIV组的中位总生存时间显着大于非TIV组（11.33年vs.4.61年； p <0.001）。使用Cox比例风险模型进行的分析表明，起病年龄和呼吸道疾病是开始TIV治疗后不良预后的独立因素。结论我们表明，接受和未接受TIV治疗的日本ALS患者的预期寿命有大约7年的显着差异。TIV组的中位总生存时间显着大于非TIV组（11.33年vs 4.61年； p <0.001）。使用Cox比例风险模型进行的分析表明，起病年龄和呼吸道疾病是开始TIV治疗后不良预后的独立因素。结论我们表明，接受和未接受TIV治疗的日本ALS患者的预期寿命有大约7年的显着差异。TIV组的中位总生存时间显着大于非TIV组（11.33年vs 4.61年； p <0.001）。使用Cox比例风险模型进行的分析表明，起病年龄和呼吸道疾病是开始TIV治疗后不良预后的独立因素。结论我们表明，接受和未接受TIV治疗的日本ALS患者的预期寿命有大约7年的显着差异。