Respiratory Rehabilitation Division, ICS Maugeri IRCCS, Lumezzane, Brescia, Italy.
NEMO Clinical Centre, Fondazione Serena Onlus, Milano, Italy.
Eur J Neurol. 2018 Mar;25(3):556-e33. doi: 10.1111/ene.13547. Epub 2018 Jan 29.
Forced vital capacity (FVC) <80% is one of the key indications for starting non-invasive ventilation (NIV) in amyotrophic lateral sclerosis (ALS). It was hypothesized that a very early start of NIV could lengthen the free interval before death compared to later-start NIV; as a secondary outcome, the survival rate of patients on NIV without tracheotomy was also evaluated.
This retrospective study was conducted on 194 ALS patients, divided into a later group (LG) with FVC <80% at NIV prescription (n = 129) and a very early group (VEG) with FVC ≥80% at NIV prescription (n = 65). Clinical and respiratory functional data and time free to death between groups over a 3-year follow-up were compared.
At 36 months from diagnosis, mortality was 35% for the VEG versus 52.7% for the LG (P = 0.022). Kaplan-Meier survival curves adjusted for tracheotomy showed a lower probability of death (P = 0.001) for the VEG as a whole (P = 0.001) and for the non-bulbar (NB) subgroup (P = 0.007). Very early NIV was protective of survival for all patients [hazard ratio (HR) 0.45; 95% confidence interval (CI) 0.28-0.74; P = 0.001] and for the NB subgroup (HR 0.43; 95% CI 0.23-0.79; P = 0.007), whilst a tracheotomy was protective for all patients (HR 0.27; 95% CI 0.15-0.50; P = 0.000) and both NB (HR 0.26; 95% CI 0.12-0.56; P = 0.001) and bulbar subgroups (HR 0.29; 95% CI 0.11-0.77; P = 0.013). Survival in VEG patients on NIV without tracheotomy was three times that for the LG (43.1% vs. 14.7%).
Very early NIV prescription prolongs the free time from diagnosis to death in NB ALS patients whilst tracheotomy reduces the mortality risk in all patients.
用力肺活量（FVC）<80%是肌萎缩侧索硬化症（ALS）开始无创通气（NIV）的关键指征之一。据推测，与晚期开始 NIV 相比，早期开始 NIV 可以延长死亡前的自由间隔期；作为次要结果，还评估了未行气管切开术的 NIV 患者的生存率。
本回顾性研究纳入了 194 例 ALS 患者，分为晚期组（LG）和非常早期组（VEG）。LG 组在 NIV 处方时 FVC<80%（n=129），VEG 组在 NIV 处方时 FVC≥80%（n=65）。比较两组患者在 3 年随访期间的临床和呼吸功能数据以及无死亡间隔时间。
从诊断起 36 个月时，VEG 组的死亡率为 35%，LG 组为 52.7%（P=0.022）。经气管切开术调整后的 Kaplan-Meier 生存曲线显示，VEG 组总体（P=0.001）和非延髓（NB）亚组（P=0.007）的死亡率较低。早期 NIV 对所有患者（危险比 [HR] 0.45；95%置信区间 [CI] 0.28-0.74；P=0.001）和 NB 亚组（HR 0.43；95% CI 0.23-0.79；P=0.007）均具有保护作用，而气管切开术对所有患者（HR 0.27；95% CI 0.15-0.50；P=0.000）和 NB 亚组（HR 0.26；95% CI 0.12-0.56；P=0.001）和延髓亚组（HR 0.29；95% CI 0.11-0.77；P=0.013）均具有保护作用。VEG 组在未行气管切开术的情况下接受 NIV 治疗的患者的生存率是 LG 组的三倍（43.1% vs. 14.7%）。
早期 NIV 处方可延长 NB 型 ALS 患者从诊断到死亡的无间隔时间，而气管切开术可降低所有患者的死亡率风险。
