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. 2017 Jul 25;18(1):144.
doi: 10.1186/s12931-017-0624-8.

The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis

T B M Tilanus  1 , J T Groothuis  2 , J M C TenBroek-Pastoor  2 , T B Feuth  3 , Y F Heijdra  4 , J P L Slenders  5 , J Doorduin  1 , B G Van Engelen  1 , M J Kampelmacher  6 , J Raaphorst  7
Affiliations

The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis

T B M Tilanus et al. Respir Res. .

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusion: 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.

Keywords: ALS; Non-invasive ventilation; Respiratory function tests.

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Conflict of interest statement

Ethics approval and consent to participate

Retrospectively designed studies do not need approval by the ethics committee, as stated by the ethics committee of the Radboud University Medical Centre.

Consent for publications

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Flowchart of the patients. The dotted box indicates the patients included for analyses. *21 patients who did not yet receive a referral to an Home Ventilation Services (HVS) or died before. NIV: Non-invasive ventilation (successfully accepted (n = 77) use >48 h), ALS: Amyotrophic Lateral Sclerosis, PMA: Progressive Muscular Atrophy
Fig. 2
Fig. 2
Time-curves of patients with 2 or more consecutive measurements. a FVC: Forced Vital Capacity (% predicted value), n = 2, 3, 9, 17, 28, 43, 67, 67; respectively. b PCF: Peak Cough Flow (L/min), n = 2, 4, 8, 10, 12, 21, 24, 24; respectively. c MIP: Maximum Inspiratory Pressure (cm H2O), n = 2, 3, 9, 15, 24, 38, 64, 64; respectively. e MEP: Maximum Expiratory Pressure (cm H2O), n = 2, 3, 8, 15, 24, 38, 64, 64; respectively. e SNIP: Sniff Nasal Inspiratory Pressure (cm H2O), n = 1, 1, 3, 3, 7, 12, 31, 31; respectively. Data are presented as mean (SD) and time intervals are presented in months before the indication of non-invasive ventilation (NIV)
Fig. 3
Fig. 3
Respiratory function tests plotted against the proportion of patients with an NIV indication within the following 3 months. a FVC: Forced Vital Capacity (% predicted value), n = 72; b PCF: Peak Cough Flow (L/min), n = 24; c MIP: Maximum Inspiratory Pressure (cm H2O), n = 70; d MEP: Maximum Expiratory Pressure (cm H2O), n = 70; e SNIP: Sniff Nasal Inspiratory Pressure (cm H2O), n = 38. NIV: Non-invasive ventilation. For example, 15% of the patients had a PCF of 386 L/min or more in the 3 months before their NIV indication
Fig. 4
Fig. 4
ROC curves. a FVC: Forced Vital Capacity (% predicted value); b PCF: Peak Cough Flow (L/min); c MIP: Maximum Inspiratory Pressure (cm H2O); d MEP: Maximum Expiratory Pressure (cm H2O); e SNIP: Sniff Nasal Inspiratory Pressure (cm H2O). The graphs are calculated with the values of the measurements of t = −3 as positive outcome and of t = −6 and t = −9 as negative outcome
See this image and copyright information in PMC

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