Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Rehabilitation, Donders Centre for Neuroscience Nijmegen, Nijmegen, The Netherlands.
Respir Res. 2017 Jul 25;18(1):144. doi: 10.1186/s12931-017-0624-8.
Non-invasive ventilation (NIV) improves survival and quality of life in amyotrophic lateral sclerosis (ALS) patients. The timing of referral to a home ventilation service (HVS), which is in part based on respiratory function tests, has shown room for improvement. It is currently unknown which respiratory function test predicts an appropriate timing of the initiation of NIV.
We analysed, retrospectively, serial data of five respiratory function tests: forced vital capacity (FVC), peak cough flow (PCF), maximum inspiratory and expiratory pressure (MIP and MEP) and sniff nasal inspiratory pressure (SNIP) in patients with ALS. Patients who had had at least one assessment of respiratory function and one visit at the HVS, were included. Our aim was to detect the test with the highest predictive value for the need for elective NIV in the following 3 months. We analysed time curves, currently used cut-off values for referral, and respiratory function test results between 'NIV indication' and 'no-NIV indication' patients.
One hundred ten patients with ALS were included of whom 87 received an NIV indication; 11.5% had one assessment before receiving an NIV indication, 88.5% had two or more assessments. The NIV indication was based on complaints of hypoventilation and/or proven (nocturnal) hypercapnia. The five respiratory function tests showed a descending trend during disease progression, where SNIP showed the greatest decline within the latest 3 months before NIV indication (mean = -22%). PCF at the time of referral to the HVS significantly discriminated between the groups 'NIV-indication' and 'no NIV-indication yet' patients at the first HVS visit: 259 (±92) vs. 348 (±137) L/min, p = 0.019. PCF and SNIP showed the best predictive characteristics in terms of sensitivity.
SNIP showed the greatest decline prior to NIV indication and PCF significantly differentiated 'NIV-indication' from 'no NIV-indication yet' patients with ALS. Currently used cut-off values might be adjusted and other respiratory function tests such as SNIP and PCF may become part of routine care in patients with ALS in order to avoid non-timely initiation of (non-invasive) ventilation.
无创通气（NIV）可提高肌萎缩侧索硬化症（ALS）患者的生存率和生活质量。转介至家庭通气服务（HVS）的时间部分基于呼吸功能测试，但该时间仍有改进空间。目前尚不清楚哪种呼吸功能测试可预测开始 NIV 的适当时间。
我们回顾性分析了 5 项呼吸功能测试的连续数据：用力肺活量（FVC）、峰值咳嗽流量（PCF）、最大吸气和呼气压力（MIP 和 MEP）以及嗅探鼻吸气压力（SNIP）在 ALS 患者中。纳入至少进行过一次呼吸功能评估和一次 HVS 就诊的患者。我们的目的是检测出对以下 3 个月内需要选择性 NIV 具有最高预测价值的测试。我们分析了“NIV 适应证”和“无 NIV 适应证”患者之间的时间曲线、目前用于转介的临界值以及呼吸功能测试结果。
共纳入 110 例 ALS 患者，其中 87 例接受了 NIV 适应证；11.5%的患者在接受 NIV 适应证前接受了一次评估，88.5%的患者接受了两次或更多次评估。NIV 适应证基于呼吸困难的主诉和/或已证实的（夜间）高碳酸血症。五项呼吸功能测试在疾病进展过程中呈下降趋势，其中 SNIP 在 NIV 适应证前最近 3 个月内下降最大（平均为-22%）。在首次 HVS 就诊时，转至 HVS 时的 PCF 显著区分了“NIV 适应证”和“尚无 NIV 适应证”患者组：259（±92）与 348（±137）L/min，p=0.019。PCF 和 SNIP 在敏感性方面表现出最佳的预测特征。
SNIP 在开始 NIV 适应证前下降最大，PCF 显著区分了“NIV 适应证”和“尚无 NIV 适应证”的 ALS 患者。目前使用的临界值可能需要调整，其他呼吸功能测试，如 SNIP 和 PCF，可能成为 ALS 患者常规护理的一部分，以避免非及时启动（无创）通气。
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