Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature
Identifieur interne : 000F65 ( Pmc/Corpus ); précédent : 000F64; suivant : 000F66Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature
Auteurs : Benedikt Schoser ; Edward Fong ; Tarekegn Geberhiwot ; Derralynn Hughes ; John T. Kissel ; Shyam C. Madathil ; David Orlikowski ; Michael I. Polkey ; Mark Roberts ; Harm A. W. M. Tiddens ; Peter YoungSource :
- Orphanet Journal of Rare Diseases [ 1750-1172 ] ; 2017.
Abstract
Respiratory muscle strength is a proven predictor of long-term outcome of neuromuscular disease (NMD), including amyotrophic lateral sclerosis, Duchenne muscular dystrophy, and spinal muscular atrophy. Maximal inspiratory pressure (MIP), a sensitive measure of respiratory muscle strength, one of several useful tests of respiratory muscle strength, is gaining interest as a therapeutic clinical trial endpoint for NMD. In this comprehensive review we investigate the use of MIP as a measure of respiratory muscle strength in clinical trials of therapeutics targeting respiratory muscle, examine the correlation of MIP with survival, quality of life, and other measures of pulmonary function, and outline the role of MIP as a clinically significantly meaningful outcome measure. Our analysis supports the utility of MIP for the early evaluation of respiratory muscle strength, especially of the diaphragm, in patients with NMD and as a surrogate endpoint in clinical trials of therapies for NMD.
Url:
DOI: 10.1186/s13023-017-0598-0
PubMed: 28302142
PubMed Central: 5353799
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PMC:5353799Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature</title><author><name sortKey="Schoser, Benedikt" sort="Schoser, Benedikt" uniqKey="Schoser B" first="Benedikt" last="Schoser">Benedikt Schoser</name><affiliation><nlm:aff id="Aff1"><institution-wrap><institution-id institution-id-type="ISNI">0000 0004 1936 973X</institution-id><institution-id institution-id-type="GRID">grid.5252.0</institution-id><institution>Friedrich-Baur-Institute, Department of Neurology,</institution><institution>Ludwig-Maximilian University,</institution></institution-wrap>Ziemssenstr. 1a, D-80336 Munich, Germany</nlm:aff></affiliation></author><author><name sortKey="Fong, Edward" sort="Fong, Edward" uniqKey="Fong E" first="Edward" last="Fong">Edward Fong</name><affiliation><nlm:aff id="Aff2"><institution-wrap><institution-id institution-id-type="ISNI">0000000419368956</institution-id><institution-id institution-id-type="GRID">grid.168010.e</institution-id><institution>Division of Pediatric Pulmonology, Asthma, and Sleep Medicine,</institution><institution>Stanford University,</institution></institution-wrap>Stanford, USA</nlm:aff></affiliation></author><author><name sortKey="Geberhiwot, Tarekegn" sort="Geberhiwot, Tarekegn" uniqKey="Geberhiwot T" first="Tarekegn" last="Geberhiwot">Tarekegn Geberhiwot</name><affiliation><nlm:aff id="Aff3">University Hospitals Birmingham, Selly Oak Hospital, Birmingham, UK</nlm:aff></affiliation></author><author><name sortKey="Hughes, Derralynn" sort="Hughes, Derralynn" uniqKey="Hughes D" first="Derralynn" last="Hughes">Derralynn Hughes</name><affiliation><nlm:aff id="Aff4"><institution-wrap><institution-id institution-id-type="ISNI">0000000121901201</institution-id><institution-id institution-id-type="GRID">grid.83440.3b</institution-id><institution>Department of Haematology,</institution><institution>Royal Free & University College Medical School,</institution></institution-wrap>London, UK</nlm:aff></affiliation></author><author><name sortKey="Kissel, John T" sort="Kissel, John T" uniqKey="Kissel J" first="John T." last="Kissel">John T. 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Tiddens</name><affiliation><nlm:aff id="Aff10"><institution-wrap><institution-id institution-id-type="GRID">grid.416135.4</institution-id><institution>Department of Pediatric Pulmonology and Allergology,</institution><institution>Sophia Children’s Hospital,</institution></institution-wrap>Rotterdam, The Netherlands</nlm:aff></affiliation></author><author><name sortKey="Young, Peter" sort="Young, Peter" uniqKey="Young P" first="Peter" last="Young">Peter Young</name><affiliation><nlm:aff id="Aff11"><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2172 9288</institution-id><institution-id institution-id-type="GRID">grid.5949.1</institution-id><institution>Department of Sleep Medicine and Neuromuscular Disorders,</institution><institution>University of Münster,</institution></institution-wrap>Münster, Germany</nlm:aff></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">28302142</idno><idno type="pmc">5353799</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5353799</idno><idno type="RBID">PMC:5353799</idno><idno type="doi">10.1186/s13023-017-0598-0</idno><date when="2017">2017</date><idno type="wicri:Area/Pmc/Corpus">000F65</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000F65</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature</title><author><name sortKey="Schoser, Benedikt" sort="Schoser, Benedikt" uniqKey="Schoser B" first="Benedikt" last="Schoser">Benedikt Schoser</name><affiliation><nlm:aff id="Aff1"><institution-wrap><institution-id institution-id-type="ISNI">0000 0004 1936 973X</institution-id><institution-id institution-id-type="GRID">grid.5252.0</institution-id><institution>Friedrich-Baur-Institute, Department of Neurology,</institution><institution>Ludwig-Maximilian University,</institution></institution-wrap>Ziemssenstr. 1a, D-80336 Munich, Germany</nlm:aff></affiliation></author><author><name sortKey="Fong, Edward" sort="Fong, Edward" uniqKey="Fong E" first="Edward" last="Fong">Edward Fong</name><affiliation><nlm:aff id="Aff2"><institution-wrap><institution-id institution-id-type="ISNI">0000000419368956</institution-id><institution-id institution-id-type="GRID">grid.168010.e</institution-id><institution>Division of Pediatric Pulmonology, Asthma, and Sleep Medicine,</institution><institution>Stanford University,</institution></institution-wrap>Stanford, USA</nlm:aff></affiliation></author><author><name sortKey="Geberhiwot, Tarekegn" sort="Geberhiwot, Tarekegn" uniqKey="Geberhiwot T" first="Tarekegn" last="Geberhiwot">Tarekegn Geberhiwot</name><affiliation><nlm:aff id="Aff3">University Hospitals Birmingham, Selly Oak Hospital, Birmingham, UK</nlm:aff></affiliation></author><author><name sortKey="Hughes, Derralynn" sort="Hughes, Derralynn" uniqKey="Hughes D" first="Derralynn" last="Hughes">Derralynn Hughes</name><affiliation><nlm:aff id="Aff4"><institution-wrap><institution-id institution-id-type="ISNI">0000000121901201</institution-id><institution-id institution-id-type="GRID">grid.83440.3b</institution-id><institution>Department of Haematology,</institution><institution>Royal Free & University College Medical School,</institution></institution-wrap>London, UK</nlm:aff></affiliation></author><author><name sortKey="Kissel, John T" sort="Kissel, John T" uniqKey="Kissel J" first="John T." last="Kissel">John T. Kissel</name><affiliation><nlm:aff id="Aff5"><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 1545 0811</institution-id><institution-id institution-id-type="GRID">grid.412332.5</institution-id><institution>Department of Neuroscience,</institution><institution>The Ohio State University Wexner Medical Center,</institution></institution-wrap>Columbus, OH USA</nlm:aff></affiliation></author><author><name sortKey="Madathil, Shyam C" sort="Madathil, Shyam C" uniqKey="Madathil S" first="Shyam C." last="Madathil">Shyam C. Madathil</name><affiliation><nlm:aff id="Aff6"><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2177 007X</institution-id><institution-id institution-id-type="GRID">grid.415490.d</institution-id><institution>Department of Respiratory Medicine,</institution><institution>University Hospital Birmingham NHS Foundation Trust, Queen Elizabeth Hospital,</institution></institution-wrap>Birmingham, England UK</nlm:aff></affiliation></author><author><name sortKey="Orlikowski, David" sort="Orlikowski, David" uniqKey="Orlikowski D" first="David" last="Orlikowski">David Orlikowski</name><affiliation><nlm:aff id="Aff7"><institution-wrap><institution-id institution-id-type="GRID">grid.414291.b</institution-id><institution>Intensive Care Unit and Home Ventilation Unit,</institution><institution>Raymond Poincare Teaching Hospital,</institution></institution-wrap>Garches, France</nlm:aff></affiliation></author><author><name sortKey="Polkey, Michael I" sort="Polkey, Michael I" uniqKey="Polkey M" first="Michael I." last="Polkey">Michael I. Polkey</name><affiliation><nlm:aff id="Aff8"><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2113 8111</institution-id><institution-id institution-id-type="GRID">grid.7445.2</institution-id><institution></institution><institution>NIHR Respiratory Biomedical Research Unit of the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London,</institution></institution-wrap>London, UK</nlm:aff></affiliation></author><author><name sortKey="Roberts, Mark" sort="Roberts, Mark" uniqKey="Roberts M" first="Mark" last="Roberts">Mark Roberts</name><affiliation><nlm:aff id="Aff9"><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 0237 2025</institution-id><institution-id institution-id-type="GRID">grid.412346.6</institution-id><institution>Greater Manchester Neurosciences Unit,</institution><institution>Salford Royal NHS Foundation Trust,</institution></institution-wrap>Salford, UK</nlm:aff></affiliation></author><author><name sortKey="Tiddens, Harm A W M" sort="Tiddens, Harm A W M" uniqKey="Tiddens H" first="Harm A. W. M." last="Tiddens">Harm A. W. M. Tiddens</name><affiliation><nlm:aff id="Aff10"><institution-wrap><institution-id institution-id-type="GRID">grid.416135.4</institution-id><institution>Department of Pediatric Pulmonology and Allergology,</institution><institution>Sophia Children’s Hospital,</institution></institution-wrap>Rotterdam, The Netherlands</nlm:aff></affiliation></author><author><name sortKey="Young, Peter" sort="Young, Peter" uniqKey="Young P" first="Peter" last="Young">Peter Young</name><affiliation><nlm:aff id="Aff11"><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2172 9288</institution-id><institution-id institution-id-type="GRID">grid.5949.1</institution-id><institution>Department of Sleep Medicine and Neuromuscular Disorders,</institution><institution>University of Münster,</institution></institution-wrap>Münster, Germany</nlm:aff></affiliation></author></analytic><series><title level="j">Orphanet Journal of Rare Diseases</title><idno type="eISSN">1750-1172</idno><imprint><date when="2017">2017</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Respiratory muscle strength is a proven predictor of long-term outcome of neuromuscular disease (NMD), including amyotrophic lateral sclerosis, Duchenne muscular dystrophy, and spinal muscular atrophy. Maximal inspiratory pressure (MIP), a sensitive measure of respiratory muscle strength, one of several useful tests of respiratory muscle strength, is gaining interest as a therapeutic clinical trial endpoint for NMD. In this comprehensive review we investigate the use of MIP as a measure of respiratory muscle strength in clinical trials of therapeutics targeting respiratory muscle, examine the correlation of MIP with survival, quality of life, and other measures of pulmonary function, and outline the role of MIP as a clinically significantly meaningful outcome measure. Our analysis supports the utility of MIP for the early evaluation of respiratory muscle strength, especially of the diaphragm, in patients with NMD and as a surrogate endpoint in clinical trials of therapies for NMD.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Perrin, C" uniqKey="Perrin C">C Perrin</name></author><author><name sortKey="Unterborn, Jn" uniqKey="Unterborn J">JN Unterborn</name></author><author><name sortKey="Ambrosio, Cd" uniqKey="Ambrosio C">CD Ambrosio</name></author><author><name sortKey="Hill, Ns" uniqKey="Hill N">NS Hill</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Epstein, Sk" uniqKey="Epstein S">SK Epstein</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Sansone, Va" uniqKey="Sansone V">VA Sansone</name></author><author><name sortKey="Gagnon, C" uniqKey="Gagnon C">C 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<front><journal-meta><journal-id journal-id-type="nlm-ta">Orphanet J Rare Dis</journal-id><journal-id journal-id-type="iso-abbrev">Orphanet J Rare Dis</journal-id><journal-title-group><journal-title>Orphanet Journal of Rare Diseases</journal-title></journal-title-group><issn pub-type="epub">1750-1172</issn><publisher><publisher-name>BioMed Central</publisher-name><publisher-loc>London</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">28302142</article-id><article-id pub-id-type="pmc">5353799</article-id><article-id pub-id-type="publisher-id">598</article-id><article-id pub-id-type="doi">10.1186/s13023-017-0598-0</article-id><article-categories><subj-group subj-group-type="heading"><subject>Review</subject></subj-group></article-categories><title-group><article-title>Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature</article-title></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Schoser</surname><given-names>Benedikt</given-names></name><address><phone>49 89 4400 57400</phone><email>bschoser@med.uni-muenchen.de</email></address><xref ref-type="aff" rid="Aff1">1</xref></contrib><contrib contrib-type="author"><name><surname>Fong</surname><given-names>Edward</given-names></name><address><email>efong@mail.cho.org</email></address><xref ref-type="aff" rid="Aff2">2</xref></contrib><contrib contrib-type="author"><name><surname>Geberhiwot</surname><given-names>Tarekegn</given-names></name><address><email>Tarekegn.Geberhiwot@uhb.nhs.uk</email></address><xref ref-type="aff" rid="Aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Hughes</surname><given-names>Derralynn</given-names></name><address><email>rmgvdah@ucl.ac.uk</email></address><xref ref-type="aff" rid="Aff4">4</xref></contrib><contrib contrib-type="author"><name><surname>Kissel</surname><given-names>John T.</given-names></name><address><email>kissel.2@osu.edu</email></address><xref ref-type="aff" rid="Aff5">5</xref></contrib><contrib contrib-type="author"><name><surname>Madathil</surname><given-names>Shyam C.</given-names></name><address><email>Shyam.Madathil@uhb.nhs.uk</email></address><xref ref-type="aff" rid="Aff6">6</xref></contrib><contrib contrib-type="author"><name><surname>Orlikowski</surname><given-names>David</given-names></name><address><email>david.orlikowski@aphp.fr</email></address><xref ref-type="aff" rid="Aff7">7</xref></contrib><contrib contrib-type="author"><name><surname>Polkey</surname><given-names>Michael I.</given-names></name><address><email>M.Polkey@rbht.nhs.uk</email></address><xref ref-type="aff" rid="Aff8">8</xref></contrib><contrib contrib-type="author"><name><surname>Roberts</surname><given-names>Mark</given-names></name><address><email>markrob@doctors.org.uk</email></address><xref ref-type="aff" rid="Aff9">9</xref></contrib><contrib contrib-type="author"><name><surname>Tiddens</surname><given-names>Harm A. W. M.</given-names></name><address><email>h.tiddens@erasmusmc.nl</email></address><xref ref-type="aff" rid="Aff10">10</xref></contrib><contrib contrib-type="author"><name><surname>Young</surname><given-names>Peter</given-names></name><address><email>Peter.Young@ukmuenster.de</email></address><xref ref-type="aff" rid="Aff11">11</xref></contrib><aff id="Aff1"><label>1</label><institution-wrap><institution-id institution-id-type="ISNI">0000 0004 1936 973X</institution-id><institution-id institution-id-type="GRID">grid.5252.0</institution-id><institution>Friedrich-Baur-Institute, Department of Neurology,</institution><institution>Ludwig-Maximilian University,</institution></institution-wrap>Ziemssenstr. 1a, D-80336 Munich, Germany</aff><aff id="Aff2"><label>2</label><institution-wrap><institution-id institution-id-type="ISNI">0000000419368956</institution-id><institution-id institution-id-type="GRID">grid.168010.e</institution-id><institution>Division of Pediatric Pulmonology, Asthma, and Sleep Medicine,</institution><institution>Stanford University,</institution></institution-wrap>Stanford, USA</aff><aff id="Aff3"><label>3</label>University Hospitals Birmingham, Selly Oak Hospital, Birmingham, UK</aff><aff id="Aff4"><label>4</label><institution-wrap><institution-id institution-id-type="ISNI">0000000121901201</institution-id><institution-id institution-id-type="GRID">grid.83440.3b</institution-id><institution>Department of Haematology,</institution><institution>Royal Free & University College Medical School,</institution></institution-wrap>London, UK</aff><aff id="Aff5"><label>5</label><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 1545 0811</institution-id><institution-id institution-id-type="GRID">grid.412332.5</institution-id><institution>Department of Neuroscience,</institution><institution>The Ohio State University Wexner Medical Center,</institution></institution-wrap>Columbus, OH USA</aff><aff id="Aff6"><label>6</label><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2177 007X</institution-id><institution-id institution-id-type="GRID">grid.415490.d</institution-id><institution>Department of Respiratory Medicine,</institution><institution>University Hospital Birmingham NHS Foundation Trust, Queen Elizabeth Hospital,</institution></institution-wrap>Birmingham, England UK</aff><aff id="Aff7"><label>7</label><institution-wrap><institution-id institution-id-type="GRID">grid.414291.b</institution-id><institution>Intensive Care Unit and Home Ventilation Unit,</institution><institution>Raymond Poincare Teaching Hospital,</institution></institution-wrap>Garches, France</aff><aff id="Aff8"><label>8</label><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2113 8111</institution-id><institution-id institution-id-type="GRID">grid.7445.2</institution-id><institution></institution><institution>NIHR Respiratory Biomedical Research Unit of the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London,</institution></institution-wrap>London, UK</aff><aff id="Aff9"><label>9</label><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 0237 2025</institution-id><institution-id institution-id-type="GRID">grid.412346.6</institution-id><institution>Greater Manchester Neurosciences Unit,</institution><institution>Salford Royal NHS Foundation Trust,</institution></institution-wrap>Salford, UK</aff><aff id="Aff10"><label>10</label><institution-wrap><institution-id institution-id-type="GRID">grid.416135.4</institution-id><institution>Department of Pediatric Pulmonology and Allergology,</institution><institution>Sophia Children’s Hospital,</institution></institution-wrap>Rotterdam, The Netherlands</aff><aff id="Aff11"><label>11</label><institution-wrap><institution-id institution-id-type="ISNI">0000 0001 2172 9288</institution-id><institution-id institution-id-type="GRID">grid.5949.1</institution-id><institution>Department of Sleep Medicine and Neuromuscular Disorders,</institution><institution>University of Münster,</institution></institution-wrap>Münster, Germany</aff></contrib-group><pub-date pub-type="epub"><day>16</day><month>3</month><year>2017</year></pub-date><pub-date pub-type="pmc-release"><day>16</day><month>3</month><year>2017</year></pub-date><pub-date pub-type="collection"><year>2017</year></pub-date><volume>12</volume><elocation-id>52</elocation-id><history><date date-type="received"><day>5</day><month>9</month><year>2016</year></date><date date-type="accepted"><day>23</day><month>2</month><year>2017</year></date></history><permissions><copyright-statement>© The Author(s). 2017</copyright-statement><license license-type="OpenAccess"><license-p><bold>Open Access</bold>This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by/4.0/">http://creativecommons.org/licenses/by/4.0/</ext-link>), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/publicdomain/zero/1.0/">http://creativecommons.org/publicdomain/zero/1.0/</ext-link>) applies to the data made available in this article, unless otherwise stated.</license-p></license></permissions><abstract id="Abs1"><p>Respiratory muscle strength is a proven predictor of long-term outcome of neuromuscular disease (NMD), including amyotrophic lateral sclerosis, Duchenne muscular dystrophy, and spinal muscular atrophy. Maximal inspiratory pressure (MIP), a sensitive measure of respiratory muscle strength, one of several useful tests of respiratory muscle strength, is gaining interest as a therapeutic clinical trial endpoint for NMD. In this comprehensive review we investigate the use of MIP as a measure of respiratory muscle strength in clinical trials of therapeutics targeting respiratory muscle, examine the correlation of MIP with survival, quality of life, and other measures of pulmonary function, and outline the role of MIP as a clinically significantly meaningful outcome measure. Our analysis supports the utility of MIP for the early evaluation of respiratory muscle strength, especially of the diaphragm, in patients with NMD and as a surrogate endpoint in clinical trials of therapies for NMD.</p></abstract><kwd-group xml:lang="en"><title>Keywords</title><kwd>Maximum inspiratory pressure</kwd><kwd>Neuromuscular disease</kwd><kwd>Respiratory failure</kwd><kwd>Endpoint</kwd><kwd>Survival</kwd><kwd>Pulmonary function testing</kwd><kwd>Spirometry</kwd></kwd-group><funding-group><award-group><funding-source><institution-wrap><institution-id institution-id-type="FundRef">http://dx.doi.org/10.13039/100008484</institution-id><institution>BioMarin Pharmaceutical</institution></institution-wrap></funding-source></award-group></funding-group><custom-meta-group><custom-meta><meta-name>issue-copyright-statement</meta-name><meta-value>© The Author(s) 2017</meta-value></custom-meta></custom-meta-group></article-meta></front><body><sec id="Sec1"><title>Background</title><p>Weakness of the respiratory muscles is especially common among patients who have an acute or chronic neuromuscular disease (NMD), including amyotrophic lateral sclerosis (ALS), Guillain-Barré syndrome, spinal muscular atrophy, myotonic dystrophy type 1, Duchenne and other muscular dystrophies, and Pompe disease [<xref ref-type="bibr" rid="CR1">1</xref>–<xref ref-type="bibr" rid="CR3">3</xref>]. In patients with a NMD, irrespective of age, pathophysiological mechanisms that lead to the development of respiratory failure frequently include progressive weakness in the inspiratory muscles, predominantly the diaphragm, as indicated by respiratory patterns with low tidal volumes [<xref ref-type="bibr" rid="CR4">4</xref>, <xref ref-type="bibr" rid="CR5">5</xref>]. However, the etiology of respiratory dysfunction can vary somewhat between different conditions [<xref ref-type="bibr" rid="CR6">6</xref>–<xref ref-type="bibr" rid="CR10">10</xref>].</p><p>NMD may impact different facets of respiratory muscle function (inspiratory, expiratory extrathoracic airways) to different extents. While expiratory muscle weakness is associated with ineffective cough, inspiratory muscle weakness causes dyspnea and/or nocturnal alveolar hypoventilation [<xref ref-type="bibr" rid="CR4">4</xref>]. Dyspnea, which results in an increased sense of effort, is a subjective sensation of breathing discomfort, likened to being smothered or suffocated. Nocturnal hypoventilation disrupts normal sleep architecture, initially in REM sleep leading to excessive daytime fatigue and morning headaches due to hypercapnia [<xref ref-type="bibr" rid="CR11">11</xref>, <xref ref-type="bibr" rid="CR12">12</xref>]. Other symptoms of nocturnal hypoventilation, include insomnia, hypersomnolence, or impaired cognition. In patients with NMD, hypoventilation during REM sleep may be an early marker of the functional impact of diaphragm weakness [<xref ref-type="bibr" rid="CR12">12</xref>]. Lastly, expiratory muscle weakness leads to ineffective clearance of airway secretions, and depending on the severity of the muscle weakness, these patients are thus at a higher risk for aspiration (more so since such patients have concomitant swallowing difficulties), bronchitis, and pneumonia [<xref ref-type="bibr" rid="CR3">3</xref>, <xref ref-type="bibr" rid="CR11">11</xref>].</p><p>The symptoms of respiratory muscle weakness can infringe significantly on daily activities (eg, walking and eating) and quality of life (QoL) of patients with NMD. Respiratory muscle weakness ultimately leads to respiratory failure and early mortality [<xref ref-type="bibr" rid="CR13">13</xref>, <xref ref-type="bibr" rid="CR14">14</xref>] or the need for noninvasive or invasive mechanical ventilation to prolong survival [<xref ref-type="bibr" rid="CR3">3</xref>, <xref ref-type="bibr" rid="CR13">13</xref>, <xref ref-type="bibr" rid="CR15">15</xref>–<xref ref-type="bibr" rid="CR18">18</xref>]. Indeed, respiratory failure secondary to muscle weakness is a common cause of premature death in NMD [<xref ref-type="bibr" rid="CR19">19</xref>]. It is, therefore, critical that patients with NMD are regularly monitored (including for measures of respiratory muscle strength and function, cough, and swallowing [<xref ref-type="bibr" rid="CR20">20</xref>, <xref ref-type="bibr" rid="CR21">21</xref>]) and subsequently managed accordingly.</p><p>Maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) are direct measures of respiratory muscle strength and may be more sensitive in detecting early respiratory muscle dysfunction compared with spirometry, but MIP and MEP are not usually performed on all patients referred for PFTs [<xref ref-type="bibr" rid="CR22">22</xref>]. MIP and MEP are noninvasive, straightforward tests in which individuals are asked to perform a forceful inspiration after an expiration to residual volume level (in the case of MIP) or expiration after a full inspiration to total lung capacity (TLC; in the case of MEP) with an open glottis against an occluded mouthpiece [<xref ref-type="bibr" rid="CR5">5</xref>, <xref ref-type="bibr" rid="CR22">22</xref>, <xref ref-type="bibr" rid="CR23">23</xref>]; the tests are generally practical in individuals older than 6 or 7 years of age. They are indicated if muscle weakness could be contributing to abnormal spirometry test results, such as a low vital capacity (VC) [<xref ref-type="bibr" rid="CR22">22</xref>]. MIP is a measure of global inspiratory muscle strength and therefore has a close relationship with diaphragmatic strength, since the diaphragm is the major inspiratory muscle; MEP is generated through the abdominal and intercostal muscles [<xref ref-type="bibr" rid="CR23">23</xref>–<xref ref-type="bibr" rid="CR26">26</xref>].</p><p>Some debate exists about a “normal” value of MIP and MEP, and different cut-off points for percentages of predicted values have been recommended, not taking into consideration any adjustments other than gender; a recent study [<xref ref-type="bibr" rid="CR27">27</xref>] has recommended using more ‘cautious’ reference equations. A 2009-update [<xref ref-type="bibr" rid="CR22">22</xref>] of the statement on respiratory muscle testing by the American Thoracic Society and the European Respiratory Society [<xref ref-type="bibr" rid="CR23">23</xref>] on the assessment of MRPs, provides more exact estimates for normal values of MIP and MEP and recommends the use of a flanged mouthpiece for the measurements. Furthermore, low MIPs can sometimes be difficult to interpret in patients with advanced illness because exertion of maximal effort is a challenge for these patients.</p><p>Together, MIP and MEP measurements can accurately assess respiratory muscle weakness, and MIP may even predict diaphragm weakness before a significant change in spirometry endpoints (eg, forced vital capacity [FVC]) [<xref ref-type="bibr" rid="CR28">28</xref>]. However, despite the potential advantages of MIP and MEP, respiratory muscle function should be evaluated with the complete array of widely available lung volume and pressure measurements, rather than relying upon individual measurements used in isolation [<xref ref-type="bibr" rid="CR29">29</xref>].</p><p>Given that respiratory muscle dysfunction—especially that of the diaphragm—is common to NMDs, then measurement of respiratory muscle strength through MIP may provide an additional meaningful endpoint in trials of therapeutics targeting respiratory muscle in patients with NMDs. With this in mind, we examined the use of MIP as a clinical endpoint in trials of therapeutics and investigated the relationship of MIP values with other parameters associated with respiratory muscle dysfunction, including survival and QoL metrics.</p></sec><sec id="Sec2"><title>Methods</title><p>We conducted literature searches in multiple databases (EMBASE, Scopus, PubMed, ProQuest, and Google Scholar; articles published before August 2015) and included clinical studies that used MIP as either a primary or secondary clinical endpoint outcome measure and reported a relationship between MIP and survival, QoL, pulmonary, and/or nonpulmonary functional endpoints. BioMarin Pharmaceutical Inc. provided funding for this analysis and for medical writing and editorial support during manuscript development. BioMarin Pharmaceutical Inc. was not involved in the collection, analysis, or interpretation of data. All authors had full access to study data and were solely responsible for the decision to submit for publication.</p><p>Search terms included the following key words: (“maximal inspiratory pressure” or “nasal inspiratory pressure” or “negative inspiratory force” or “MIP” or “PImax”) AND (“outcome” or “endpoint” or “efficacy” or “treatment effectiveness” or “sleep-disordered breathing” or “nocturnal hypoventilation” or “mortality” or “survival” or “death”). We initially screened literature records on the basis of title and abstract and excluded records not meeting the inclusion criteria (eg, no MIP results reported, disease and/or natural history focused).</p><p>To identify open, ongoing clinical trials that are using MIP as an endpoint (primary or secondary), we searched the ClinicalTrials.gov database (<ext-link ext-link-type="uri" xlink:href="http://www.clinicaltrials.gov/">www.clinicaltrials.gov</ext-link>). The search was conducted on July 12, 2015; search terms included <italic>maximal inspiratory pressure</italic>, <italic>PImax</italic>, <italic>and negative inspiratory force</italic>.</p></sec><sec id="Sec3"><title>Results</title><sec id="Sec4"><title>MIP as an endpoint in clinical trials</title><p>We identified 8 publications in which MIP was used as a primary or secondary endpoint in a randomized controlled trial (RCT) of a pharmacologic therapy in a broad spectrum of conditions (Table <xref rid="Tab1" ref-type="table">1</xref>) [<xref ref-type="bibr" rid="CR26">26</xref>, <xref ref-type="bibr" rid="CR30">30</xref>–<xref ref-type="bibr" rid="CR36">36</xref>]. One of these studies was in patients with Duchenne muscular dystrophy [<xref ref-type="bibr" rid="CR36">36</xref>]. Across the studies, the MIP endpoint was reported to be a sensitive and specific clinical measure for evaluation of the pharmacologic interventions, all of which directly targeted the respiratory musculature.<table-wrap id="Tab1"><label>Table 1</label><caption><p>Completed RCTs using MIP as a clinical endpoint</p></caption><table frame="hsides" rules="groups"><thead><tr><th>Reference</th><th>MIP Endpoint</th><th>Treatment Group</th><th>N</th><th>MIP Result</th></tr></thead><tbody><tr><td>Golparvar M et al., 2005 [<xref ref-type="bibr" rid="CR30">30</xref>]</td><td>Primary</td><td>Progesterone administration in adult trauma patients during partial support ventilation</td><td>40</td><td>MIP significantly increased (<italic>P</italic> < 0.05) 3 h after administration</td></tr><tr><td>Gontijo-Amaral C et al., 2012 [<xref ref-type="bibr" rid="CR26">26</xref>]</td><td>Primary</td><td>Oral magnesium supplementation in adolescent cystic fibrosis patients</td><td>44</td><td>Significant increase in MIP between intervention and placebo period (<italic>P</italic> < 0.001)</td></tr><tr><td>Mackersie RC et al., 1991 [<xref ref-type="bibr" rid="CR31">31</xref>]</td><td>Primary</td><td>Continuous epidural or continual IV infusions of fentanyl in patients with multiple rib fractures</td><td>32</td><td>Significant increase in MIP in epidural and fentanyl epidural groups compared with pre-analgesia (<italic>P</italic> < 0.05)</td></tr><tr><td>Sosis M et al., 1987 [<xref ref-type="bibr" rid="CR32">32</xref>]</td><td>Primary</td><td>Atracurium treatment in patients requiring intubation</td><td>39</td><td>Significant decrease in MIP in patients receiving atracurium compared with placebo (<italic>P</italic> < 0.05)</td></tr><tr><td>Andreas S et al., 2006 [<xref ref-type="bibr" rid="CR35">35</xref>]</td><td>Primary</td><td>Irbesartan treatment in COPD patients</td><td>60</td><td>No significant difference in MIP after 4 months of treatment (<italic>P</italic> = 0.16). Reporting a large standard deviation of MIP.</td></tr><tr><td>Skorodin MS et al., 1995 [<xref ref-type="bibr" rid="CR33">33</xref>]</td><td>Primary</td><td>Magnesium sulphate administration in COPD patients</td><td>72</td><td>No significant difference in MIP after 20 and 45 min of treatment (<italic>P</italic> = NS)</td></tr><tr><td>Weisberg J et al., 2002 [<xref ref-type="bibr" rid="CR34">34</xref>]</td><td>Primary</td><td>Megestrol acetate administration in COPD patients</td><td>128</td><td>No significant difference in MIP (<italic>P</italic> = NS). Reporting a large standard deviation of MIP.</td></tr><tr><td>Buyse GM et al., 2013 [<xref ref-type="bibr" rid="CR36">36</xref>]</td><td>Secondary</td><td>Idebenone treatment in Duchenne muscular dystrophy patients</td><td>21</td><td>MIP improved in idebenone group but deteriorated in placebo group (<italic>P</italic> = NS)</td></tr></tbody></table><table-wrap-foot><p><italic>COPD</italic> chronic obstructive pulmonary disease, <italic>IV</italic> intravenous, <italic>MIP</italic> maximum inspiratory pressure, <italic>NS</italic> not significant</p></table-wrap-foot></table-wrap></p><p>A search of the clinical trials database revealed 31 open trials where MIP was cited as either a primary or secondary endpoint (Table <xref rid="Tab2" ref-type="table">2</xref>). The trials reflected a range of clinical conditions; approximately 1 in 3 were in individuals with a NMD (including ALS, Duchenne muscular dystrophy, myasthenia gravis, Pompe disease, and X-linked myotubular myopathy). This suggests that MIP is gaining momentum as clinical endpoint for monitoring respiratory muscle function in these patients.<table-wrap id="Tab2"><label>Table 2</label><caption><p>Ongoing<sup>a</sup> clinical trials with MIP as an endpoint</p></caption><table frame="hsides" rules="groups"><thead><tr><th>NCT number</th><th>Study Title</th><th>Study Design</th><th>Primary or Secondary Endpoint</th><th>Estimated Study Completion Date</th><th>Estimated Enrollment</th></tr></thead><tbody><tr><td colspan="6">Neuromuscular diseases</td></tr><tr><td colspan="6"> Amyotrophic lateral sclerosis</td></tr><tr><td> NCT02478450</td><td>Study to Investigate the Safety of the Transplantation (by Injection) of Human Glial Restricted Progenitor Cells (hGRPs; Q-Cells®) Into Subjects With Amyotrophic Lateral Sclerosis (ALS)</td><td>Nonrandomized, open-label, parallel group study</td><td>Secondary</td><td>December 2017</td><td>12</td></tr><tr><td colspan="6">Duchenne muscular dystrophy</td></tr><tr><td> NCT02310906</td><td>Phase I/II Study of SRP-4053 in DMD Patients</td><td>Randomized, placebo-controlled, double-blind, parallel group study</td><td>Secondary</td><td>December 2016</td><td>48</td></tr><tr><td> NCT01999075</td><td>Stacking Exercises Aid the Decline in FVC and Sick Time (STEADFAST)</td><td>Randomized, single-blind, parallel group study</td><td>Secondary</td><td>August 2016</td><td>110</td></tr><tr><td> NCT02255552</td><td>Confirmatory Study of Eteplirsen in DMD Patients (PROMOVI)</td><td>Nonrandomized, open-label, parallel group study</td><td>Secondary</td><td>May 2016</td><td>160</td></tr><tr><td colspan="6">Myasthenia gravis</td></tr><tr><td> NCT01047761</td><td>Exercise for Stable Myasthenia Gravis</td><td>Nonrandomized, open-label, single group study</td><td>Secondary</td><td>December 2015</td><td>30</td></tr><tr><td colspan="6">Parkinson’s disease</td></tr><tr><td> NCT02202057</td><td>Respiratory Load Magnitude Estimation in PD</td><td>Prospective, case control study</td><td>Secondary</td><td>August 2015</td><td>80</td></tr><tr><td colspan="6">Pompe disease</td></tr><tr><td> NCT02357225</td><td>A Pilot Study of Pyridostigmine in Pompe Disease</td><td>Nonrandomized, open-label, single group study</td><td>Primary</td><td>June 2017</td><td>16</td></tr><tr><td> NCT02354651</td><td>Response to Diaphragmatic Pacing in Subjects With Pompe Disease</td><td>Prospective, observational study</td><td>Secondary</td><td>February 2017</td><td>12</td></tr><tr><td> NCT01924845</td><td>BMN 701 Phase 3 in rhGAA Exposed Subjects With Late Onset Pompe Disease (INSPIRE Study)</td><td>Nonrandomized, open-label, single group study</td><td>Primary</td><td>June 2020</td><td>50</td></tr><tr><td colspan="6">Pulmonary hypertension</td></tr><tr><td> NCT02288442</td><td>Whole Muscle Exercise Training (WHOLEi + 12) in Pulmonary Hypertension</td><td>Randomized, single-blind, parallel group study</td><td>Secondary</td><td>September 2016</td><td>20</td></tr><tr><td colspan="6">Sarcopenia</td></tr><tr><td> NCT02120586</td><td>Preventive Physiotherapy Intervention in Elderly People With Sarcopenia</td><td>Randomized, single-blind, parallel group study</td><td>Primary</td><td>July 2015</td><td>70</td></tr><tr><td colspan="6">X-linked myotubular myopathy</td></tr><tr><td> NCT02453152</td><td>Respiratory Muscle Function in Untreated X-Linked Myotubular Myopathy (XLMTM)</td><td>Prospective, observational study</td><td>Secondary</td><td>December 2016</td><td>12</td></tr><tr><td colspan="6">Other disorders (non-NMDs)</td></tr><tr><td colspan="6"> Back pain</td></tr><tr><td> NCT02429752</td><td>Low Back Pain and Breathing Pattern Dysfunction (LBP & BPD)</td><td>Open-label, single group study</td><td>Secondary</td><td>June 2016</td><td>75</td></tr><tr><td colspan="6">Breast disease</td></tr><tr><td> NCT02491762</td><td>The Effect of Breast Reconstruction Surgery Using Tissue Expanders on Respiratory Functions</td><td>Nonrandomized, open-label, parallel group study</td><td>Primary</td><td>August 2017</td><td>45</td></tr><tr><td> NCT02165696</td><td>Compression Bandaging and Manual Lymph Drainage in Women With Lymphedema (LYMPHATIC)</td><td>Randomized, single-blind, parallel group study</td><td>Secondary</td><td>December 2015</td><td>44</td></tr><tr><td colspan="6">COPD</td></tr><tr><td> NCT01655199</td><td>Sensitivity of the Step Test to Detect Improvement in Dyspnea Following Bronchodilation in Patients With Chronic Obstructive Pulmonary Disease (CODEx)</td><td>Double-blind, single group study</td><td>Secondary</td><td>July 2014</td><td>40</td></tr><tr><td> NCT01903772</td><td>Effects of Inspiratory Muscle Training in Chronic Obstructive Pulmonary Disease (COPD) (IMTCO)</td><td>Randomized, double-blind, parallel group study</td><td>Secondary</td><td>December 2015</td><td>72</td></tr><tr><td> NCT01956565</td><td>Feasibility of Inspiratory Muscle Training in People With COPD Who Decline Pulmonary Rehabilitation</td><td>Nonrandomized, open-label, single group study</td><td>Secondary</td><td>November 2015</td><td>20</td></tr><tr><td> NCT02392715</td><td>Inspiratory Muscle Training Combined With General Exercise Training in COPD (IMTGET)</td><td>Randomized, double-blind, parallel group study</td><td>Secondary</td><td>October 2016</td><td>80</td></tr><tr><td> NCT02007772</td><td>Effectiveness of TNI vs. BiPAP in Chronic Global Insufficiency in COPD Patients (TIBICO)</td><td>Randomized, open-label, crossover study</td><td>Secondary</td><td>August 2015</td><td>85</td></tr><tr><td> NCT01582958</td><td>The Effect of OMT on Patients With COPD: Correlating Pulmonary Function Tests With Biochemical Alterations</td><td>Randomized, single-blind, parallel group study</td><td>Primary</td><td>August 2015</td><td>60</td></tr><tr><td> NCT01037387</td><td>Effect of Noninvasive Ventilation on Physical Activity and Inflammation in COPD Patients</td><td>Randomized, open-label, parallel group study</td><td>Secondary</td><td>June 2016</td><td>50</td></tr><tr><td colspan="6">COPD or asthma</td></tr><tr><td> NCT02233114</td><td>Do Yogic Exercises (12 weeks) Increase Respiratory Function in Patients with Obstructive Lung Diseases?</td><td>Randomized, single-blind, parallel group study</td><td>Secondary</td><td>December 2017</td><td>40</td></tr><tr><td colspan="6">Chronic respiratory insufficiency</td></tr><tr><td> NCT01458314</td><td>Non Invasive Mechanical Ventilation in Chronic Respiratory Insufficiency Patients During Rehabilitation</td><td>Randomized, open-label, parallel group study</td><td>Secondary</td><td>June 2015</td><td>50</td></tr><tr><td colspan="6">Chronic respiratory failure</td></tr><tr><td> NCT00994552</td><td>Comparison of Pressure Support and Pressure Control Ventilation in Chronic Respiratory Failure</td><td>Randomized, single-blind crossover study</td><td>Secondary</td><td>April 2010</td><td>20</td></tr><tr><td colspan="6">Congenital heart disease</td></tr><tr><td> NCT02438293</td><td>The Impact of Rhinovirus Infections in Paediatric Cardiac Surgery’ (RISK)</td><td>Prospective, observational study</td><td>Secondary</td><td>June 2016</td><td>250</td></tr><tr><td colspan="6">Intraventricular hemorrhage</td></tr><tr><td> NCT02231411</td><td>Neonatal Resuscitation With Intact Cord (NRIC)</td><td>Randomized, single-blind, parallel group study</td><td>Secondary</td><td>August 2016</td><td>150</td></tr><tr><td colspan="6">Lung cancer</td></tr><tr><td> NCT02493114</td><td>Functional Status in Patients Undergoing Curative Treatment for Lung Cancer</td><td>Prospective, observational study</td><td>Secondary</td><td>November 2017</td><td>80</td></tr><tr><td colspan="6">Mechanically ventilated patients</td></tr><tr><td> NCT02003053</td><td>A Randomized, Controlled Trial of Inspiratory Muscle Training (IMT) in the ICU and CCU</td><td>Randomized, single-blind, parallel group study</td><td>Primary</td><td>September 2015</td><td>40</td></tr><tr><td colspan="6">Obstructive sleep apnea</td></tr><tr><td> NCT02259660</td><td>Airway Muscle Training for Obstructive Sleep Apnea (OSA)</td><td>Randomized, double-blind, parallel group study</td><td>Secondary</td><td>September 2016</td><td>60</td></tr><tr><td colspan="6">Stroke</td></tr><tr><td> NCT02400138</td><td>Home-based Respiratory Training After Stroke</td><td>Randomized, double-blind, parallel group study</td><td>Primary</td><td>November 2016</td><td>20</td></tr></tbody></table><table-wrap-foot><p><sup>a</sup>Clinical trials in progress as of October 31<sup>st</sup>, 2015</p><p><italic>BiPAP</italic> biphasic positive airway pressure, <italic>CCU</italic> critical care unit, <italic>COPD</italic> chronic obstructive pulmonary disease, <italic>DMD</italic> Duchenne muscular dystrophy, <italic>FVC</italic> forced vital capacity, <italic>ICU</italic> intensive care unit, <italic>MIP</italic> maximum inspiratory pressure, <italic>OMT</italic> osteopathic manipulative treatment, <italic>PD</italic> Parkinson’s disease, <italic>TNI</italic> therapy with nasal insufflation</p></table-wrap-foot></table-wrap></p></sec><sec id="Sec5"><title>Relationship between MIP and survival in patients with NMD</title><p>As noted, respiratory failure is a common cause of premature death in patients with NMD [<xref ref-type="bibr" rid="CR19">19</xref>]. Consequently, patients with progressive disease require frequent monitoring of their pulmonary function. Sensitive, noninvasive predictive measures are needed to quantify the risk of mortality due to respiratory failure in these patients; predictive measures could also quantify the potential mortality risk benefit of a therapeutic intervention.</p><p>Studies have investigated the correlation between MIP and survival in various conditions (Table <xref rid="Tab3" ref-type="table">3</xref>) [<xref ref-type="bibr" rid="CR37">37</xref>–<xref ref-type="bibr" rid="CR51">51</xref>]. In regard to NMD, the majority of data comes from patients with ALS. All ALS published studies we identified consistently found a correlation between MIP and survival [<xref ref-type="bibr" rid="CR37">37</xref>–<xref ref-type="bibr" rid="CR41">41</xref>]. A cohort study in 95 patients with ALS found a significant association between MIP and 1-year survival (<italic>P</italic> < 0.05) [<xref ref-type="bibr" rid="CR37">37</xref>]. The study found that, whereas a normal (>80% predicted) supine FVC predicted a > 80% chance of 1-year tracheostomy-free survival, a normal MIP or MEP predicted a > 90% chance of survival. In a second ALS study, reduced MIP predicted poor 2-years survival. Extensively controlled for nonpulmonary factors known to predict survival in ALS, Kaplan-Meier and receiver operating characteristic curve analysis showed that 2-years survival was more probable in patients with initially normal MIP values (<italic>P</italic> = 0.0001) compared with patients who had initially reduced MIP (<70 cm H<sub>2</sub>O; <italic>P</italic> < 0.05) [<xref ref-type="bibr" rid="CR38">38</xref>]. In a third study of ALS patients (<italic>N</italic> = 21), MIP (−60 cm H<sub>2</sub>O or less) was 100% sensitive as a “threshold” for predicting 18-months survival, whereas FVC (<80% of predicted) was not as sensitive for predicting survival (<80% sensitive) [<xref ref-type="bibr" rid="CR39">39</xref>]. In a fourth study of 53 patients with ALS, comparison of baseline data in patients who were dead or alive at 18 months showed that survivors had a higher mean MIP (38 ± 24% predicted) than nonsurvivors (20 ± 18% predicted; <italic>P</italic> < 0.01). The absence of cough spikes (defined as peak flow rate transients during voluntary cough) had no significant influence on survival [<xref ref-type="bibr" rid="CR40">40</xref>]. Finally, clinical results from a 5-years prospective, comparative trial of patients with ALS using noninvasive ventilation found that determinants of respiratory function (including MIP [<italic>P</italic> = 0.0001]) were an independent predictor of 5-years survival, emphasizing the potential utility of MIP as a prognostic indicator in patients with ALS [<xref ref-type="bibr" rid="CR41">41</xref>].<table-wrap id="Tab3"><label>Table 3</label><caption><p>Summary of studies investigating the correlation between MIP and survival</p></caption><table frame="hsides" rules="groups"><thead><tr><th>Reference</th><th>Therapy Area/Population</th><th>Study Type</th><th>N</th><th>MIP and Survival</th></tr></thead><tbody><tr><td colspan="5">Correlation between MIP and survival</td></tr><tr><td> Schmidt EP et al., 2006 [<xref ref-type="bibr" rid="CR37">37</xref>]</td><td>ALS</td><td>Cohort study</td><td>95</td><td>MIP was an important predictor of 1-year survival (<italic>P</italic> < 0.05) after controlling for nonpulmonary factors known to predict survival in ALS</td></tr><tr><td> Baumann F et al., 2010 [<xref ref-type="bibr" rid="CR38">38</xref>]</td><td>ALS</td><td>Cohort study</td><td>80</td><td>MIP was significantly associated with survival (<italic>P</italic> < 0.05); survival time was increased in patients with normal MIP</td></tr><tr><td> Gay PC et al., 1991 [<xref ref-type="bibr" rid="CR39">39</xref>]</td><td>ALS</td><td>Prospective study</td><td>21</td><td>MIP was 100% sensitive for predicting 18-months survival (<italic>r</italic> = 0.57; <italic>P</italic> < 0.007)</td></tr><tr><td> Chaudri MB et al., 2002 [<xref ref-type="bibr" rid="CR40">40</xref>]</td><td>MND (ALS)</td><td>Single-center cohort study</td><td>53</td><td>Survivors had a higher percentage of predicted MIP than nonsurvivors (37.83 ± 24.32% vs 20.13 ± 18.43%; <italic>P</italic> < 0.01)</td></tr><tr><td> Lopes Almeida JP, et al., 2012 [<xref ref-type="bibr" rid="CR41">41</xref>]</td><td>ALS</td><td>Prospective, comparative study</td><td>60</td><td>There was a significant correlation between MIP and 5-years survival (<italic>P</italic> = 0.02) in patients with ALS using NIV</td></tr><tr><td> Benzo R et al., 2013 [<xref ref-type="bibr" rid="CR42">42</xref>]</td><td>COPD</td><td>Analysis of clinical data from NETT</td><td>1218</td><td>Decrease in MIP > 11 cm H<sub>2</sub>O was a predictor of 1-year mortality (OR, 2.19; <italic>P</italic> = 0.0217)</td></tr><tr><td> Gray-Donald K et al., 1996 [<xref ref-type="bibr" rid="CR43">43</xref>]</td><td>COPD</td><td>3 to 5-years follow-up of a double-blind RCT</td><td>348</td><td>Low MIP was a significant independent predictor of respiratory (HR, 0.64; 95% CI, 0.44–0.95) and all-cause mortality (HR, 0.67; 95% CI, 0.47–0.95)</td></tr><tr><td> Schols AMWJ et al., 1998 [<xref ref-type="bibr" rid="CR44">44</xref>]</td><td>COPD</td><td>RCT</td><td>203</td><td>Improvement in MIP during rehabilitation decreased the risk of death</td></tr><tr><td> Hodgev VA et al., 2006 [<xref ref-type="bibr" rid="CR45">45</xref>]</td><td>COPD</td><td>Prospective cohort study</td><td>63</td><td>A Cox proportional hazards analysis showed that MIP was a significant predictor of mortality (<italic>r</italic> = 0.91; 95% CI, 0.85–0.97; <italic>P</italic> = 0.005)</td></tr><tr><td> Meyer FJ et al., 2001 [<xref ref-type="bibr" rid="CR47">47</xref>]</td><td>Heart failure</td><td>Prospective study</td><td>244</td><td>In a univariate Cox regression analysis, MIP was found to be a significant prognostic indicator of survival (<italic>P</italic> = 0.001)</td></tr><tr><td> Frankenstein L et al., 2009 [<xref ref-type="bibr" rid="CR48">48</xref>]</td><td>Heart failure</td><td>Prospective, observational study</td><td>686</td><td>MIP was identified as a significant predictor of survival by univariate analysis; survivors had a significantly higher MIP and percentage of predicted MIP than nonsurvivors</td></tr><tr><td> Ionescu AA et al., 1998 [<xref ref-type="bibr" rid="CR49">49</xref>]</td><td>Cystic fibrosis</td><td>Single-center study</td><td>49</td><td>Mean % predicted MIP (SD) for survivors was 85.5% (28.4) compared with 64.1% (23.9) for nonsurvivors</td></tr><tr><td> Marroni CA et al., 2014 [<xref ref-type="bibr" rid="CR50">50</xref>]</td><td>Liver cirrhosis</td><td>Prospective cohort study</td><td>86</td><td>Sixty-two percent of patients with MIP < −70 cm H<sub>2</sub>O survived compared with 93% of patients with MIP > −70 cm H<sub>2</sub>O (<italic>P</italic> = 0.0001)</td></tr><tr><td> Budweiser S et al., 2007 [<xref ref-type="bibr" rid="CR46">46</xref>]</td><td>Chronic hypercapnic respiratory failure</td><td>Cohort study</td><td>464</td><td>MIP was a significant predictor of long-term survival; according to stepwise multivariate Cox regression analysis, P<sub>0.1</sub>/MIP was identified as an independent predictor of survival (<italic>P</italic> < 0.05)</td></tr><tr><td> van der Palen J et al., 2004 [<xref ref-type="bibr" rid="CR51">51</xref>]</td><td>Elderly with a mean age of 72.5 years</td><td>Cohort study</td><td>3839</td><td>Subjects in the lowest quintile of MIP had a 1.5-fold increased risk of cardiovascular-related death (HR, 1.54; 95% CI, 1.09–2.15) after adjustment for nonpulmonary function covariates</td></tr><tr><td colspan="5">No correlation between MIP and survival</td></tr><tr><td> Nizet TAC et al., 2005 [<xref ref-type="bibr" rid="CR58">58</xref>]</td><td>Chronic hypercapnic COPD</td><td>Prospective, single-center study</td><td>47</td><td>No significant association</td></tr><tr><td> Frankenstein L et al., 2008 [<xref ref-type="bibr" rid="CR54">54</xref>]</td><td>CHF</td><td>Prospective, single-arm study</td><td>158</td><td>No significant association</td></tr><tr><td> Habedank D et al., 2013 [<xref ref-type="bibr" rid="CR55">55</xref>]</td><td>CHF</td><td>Single-center study</td><td>249</td><td>No significant association</td></tr><tr><td> Hui D et al., 2014 [<xref ref-type="bibr" rid="CR56">56</xref>]</td><td>Advanced cancer</td><td>Prospective, single-center study</td><td>222</td><td>No significant association</td></tr><tr><td> White AC et al., 2005 [<xref ref-type="bibr" rid="CR59">59</xref>]</td><td>Hematopoietic stem cell transplantation</td><td>Prospective observational study</td><td>56</td><td>No significant association</td></tr><tr><td> Jackson M et al., 1994 [<xref ref-type="bibr" rid="CR57">57</xref>]</td><td>Patients with a thoracoplasty for tuberculosis</td><td>Single-center study</td><td>32</td><td>No significant association</td></tr></tbody></table><table-wrap-foot><p><italic>ALS</italic> amyotrophic lateral sclerosis, <italic>CHF</italic> congestive heart failure, <italic>CI</italic> confidence interval, <italic>COPD</italic> chronic obstructive pulmonary disease, <italic>HR</italic> hazard ratio, <italic>MIP</italic> maximum inspiratory pressure, <italic>MND</italic> motor neurone disease, <italic>NETT</italic> National Emphysema Treatment Trial, <italic>NIV</italic> noninvasive ventilation, <italic>OR</italic> odds ratio, <italic>P</italic><sub><italic>0.1</italic></sub> mouth occlusion pressure, <italic>RCT</italic> randomized controlled trial, <italic>SD</italic> standard deviation</p></table-wrap-foot></table-wrap></p><p>In a further study recently published by one of the current authors [<xref ref-type="bibr" rid="CR52">52</xref>], multiple outcome measures were obtained in 78 patients with ALS who were then followed until death. Low values for MIP were highly specific predictors of time to death or initiation of NIV; conversely, while VC was also a specific predictor, the cut points suggested by ROC analysis were >80% of normal at all time points except for 3 months mortality prediction (when it was 78%), suggesting that a normal VC was of limited practical value. However, a small (<italic>N</italic> = 18) study of MIP and survival in patients with Duchenne muscular dystrophy [<xref ref-type="bibr" rid="CR53">53</xref>] did not find a predictive association. It should be noted that participants had extremely low values of MIP and VC at the start of study and that the analysis did not include the use of noninvasive ventilation.</p><p>Some studies in non-neuromuscular diseases—chronic obstructive pulmonary disease (COPD) [<xref ref-type="bibr" rid="CR42">42</xref>–<xref ref-type="bibr" rid="CR45">45</xref>], cystic fibrosis [<xref ref-type="bibr" rid="CR49">49</xref>], liver cirrhosis [<xref ref-type="bibr" rid="CR50">50</xref>], hypercapnic respiratory failure [<xref ref-type="bibr" rid="CR46">46</xref>], and congestive heart failure [<xref ref-type="bibr" rid="CR47">47</xref>, <xref ref-type="bibr" rid="CR48">48</xref>, <xref ref-type="bibr" rid="CR51">51</xref>]—suggest that MIP may correlate with survival, while others report no correlation [<xref ref-type="bibr" rid="CR54">54</xref>–<xref ref-type="bibr" rid="CR59">59</xref>] (Table <xref rid="Tab3" ref-type="table">3</xref>), indicating that further investigation is needed. We also caution that in non-neuromuscular disease states, a reduced MIP may simply reflect generalized cachexia, which is a recognized marker of a poor prognosis and hyperinflation [<xref ref-type="bibr" rid="CR60">60</xref>] in pulmonary disease.</p></sec><sec id="Sec6"><title>Relationship between MIP and QoL in patients with NMD</title><p>As NMD pathology progresses and patients develop respiratory dysfunction, QoL (with respect to a patient’s physical, emotional, social functioning, mental health, bodily pain, endurance, and general health perceptions) and sleep can be severely impacted [<xref ref-type="bibr" rid="CR18">18</xref>, <xref ref-type="bibr" rid="CR61">61</xref>, <xref ref-type="bibr" rid="CR62">62</xref>]. The relationship between changes in MIP and QoL in NMD was addressed in 2 studies from our literature search: one in ALS [<xref ref-type="bibr" rid="CR18">18</xref>] and one in patients with post-poliomyelitis syndrome [<xref ref-type="bibr" rid="CR63">63</xref>].</p><p>Bourke et al. evaluated the impact of noninvasive intervention on QoL in 22 patients with ALS using the 36-Item Short Form (SF-36) and the National Center for Health Statistics General Well-Being Schedule and concluded that respiratory muscle weakness had an impact on QoL [<xref ref-type="bibr" rid="CR18">18</xref>]. Overall, the researchers found that patients with ALS with significantly lower QoL displayed lower MIP values. Lower MIP values corresponded with lower SF-36 scores in all domains except the pain and physical components [<xref ref-type="bibr" rid="CR18">18</xref>]. Instruments specifically assessing respiratory and sleep-related problems (eg, the Epworth Sleepiness Scale [ESS]) were most sensitive to changes in MIP [<xref ref-type="bibr" rid="CR18">18</xref>]. In this regard, ESS scores were highest (indicating sleep disruption) in patients with MIP values below 50% and were lowest in patients with MIP values above 50% (10.3 and 4.8%, respectively; <italic>P</italic> = 0.01) [<xref ref-type="bibr" rid="CR18">18</xref>].</p><p>Similar to the findings of Bourke et al., a cross-sectional study of 52 patients with post-poliomyelitis syndrome observed a significant correlation between MIP and both the fatigue severity scale and the Multidimensional Fatigue Inventory (MFI) (<italic>r</italic> = −0.31 and <italic>r</italic> = −0.41, respectively; <italic>P</italic> < 0.05). The researchers also found that a 10-unit decline for MIP (% predicted) corresponded to patients scoring a 0.3-unit increase on the General Fatigue dimension of the MFI scale [<xref ref-type="bibr" rid="CR63">63</xref>]. This dimension of the MFI scale ranges from 4 to 20, with higher scores indicating more severe fatigue.</p></sec><sec id="Sec7"><title>Relationship between MIP and spirometry in patients with NMD</title><p>In patients with NMD whose lungs are restricted from fully expanding, spirometry is widely used to assess respiratory muscle function. Patients performing spirometry are asked to take a maximal inspiration and perform a FVC maneuver. A drop of more than 20% of FVC going from the upright to the supine position is a useful diagnostic of diaphragmatic weakness [<xref ref-type="bibr" rid="CR64">64</xref>]. FVC, however, has a curvilinear relationship with respiratory muscle strength, and substantial weakness may be present while FVC is still within the normal range [<xref ref-type="bibr" rid="CR65">65</xref>].</p><p>Lung volume measurements are sometimes performed in patients with NMD, including TLC and functional residual capacity (FRC; defined as the amount of air in the lungs following normal expiration). In these patients, when inspiratory muscles are weak, then a maximum effort may be insufficient to fully expand the lungs, and the TLC and FRC will be reduced [<xref ref-type="bibr" rid="CR22">22</xref>, <xref ref-type="bibr" rid="CR24">24</xref>]. Similarly, if abdominal muscle strength is impaired, residual volume may also be elevated [<xref ref-type="bibr" rid="CR22">22</xref>].</p><p>When carbon monoxide gas transfer is measured, the classic picture of respiratory muscle weakness is a low diffusion capacity (DL<sub>CO</sub>) and an elevated transfer coefficient (K<sub>CO</sub>). However, a study in patients with NMDs showed that the rise in K<sub>CO</sub> was often less than expected; in patients with combined inspiratory and expiratory muscle weakness, a reduced value was observed [<xref ref-type="bibr" rid="CR66">66</xref>]. The results demonstrated the limitations of using K<sub>CO</sub> in the diagnosis of respiratory muscle weakness. Gas exchange anomalies can also be multifactorial in their origin, for example, mechanical problems and airway obstructions can affect results [<xref ref-type="bibr" rid="CR67">67</xref>].</p><p>While the above discussed spirometry tests have utility, they can be considered insensitive measures of respiratory muscle function since a significant reduction in lung volume may not be observed until severe impairment of respiratory muscles has occurred [<xref ref-type="bibr" rid="CR67">67</xref>]. Also, other factors such as airway obstruction due to asthma or lower airway obstruction may affect the reliability of some spirometry results [<xref ref-type="bibr" rid="CR5">5</xref>, <xref ref-type="bibr" rid="CR68">68</xref>–<xref ref-type="bibr" rid="CR70">70</xref>]. Consistent with these limitations, when measured within 30 days of the need for tracheostomy in a clinical trial of therapeutics for ALS [<xref ref-type="bibr" rid="CR71">71</xref>], VC was ≥ 60% predicted in 14% of ALS patients (<italic>n</italic> = 50).</p><p>MIP has physiological relationships with spirometry endpoints [<xref ref-type="bibr" rid="CR65">65</xref>]. Specifically, in patients with a NMD, several studies have shown a correlation between MIP and FVC. In a study of patients with Duchenne muscular dystrophy, a significant 1-year decrease in MIP was associated with decreases in FVC, FEV<sub>1</sub> (defined as forced expiratory volume in 1 sec), and peak expiratory flow rate (<italic>P</italic> < 0.05 for all measures) [<xref ref-type="bibr" rid="CR72">72</xref>]. Similarly, Schmidt et al. found a significant association between MIP and upright FVC in a 1-year cohort study of 95 patients with ALS [<xref ref-type="bibr" rid="CR37">37</xref>].</p><p>Also, the LOTS study investigated the effect of alglucosidase alfa treatment in patients with late-onset Pompe disease [<xref ref-type="bibr" rid="CR73">73</xref>]. Patients who entered an open-label extension phase of this trial showed additional improvement in MIP but a slight decline in FVC from week 78 through week 104; a statistical correlation however was not reported [<xref ref-type="bibr" rid="CR74">74</xref>]. Finally, a RCT conducted by Cheah et al. to assess the effects of a 12-weeks inspiratory muscle training program in patients with ALS found that improvements in MIP reflected improvements in FVC and TLC [<xref ref-type="bibr" rid="CR75">75</xref>]. In 3 RCTs where MIP improvements were observed following pharmacologic treatment in patients with non-neuromuscular diseases, these values also correlated with improvements in other pulmonary measures, including FVC (as well as airway resistance and MEP) [<xref ref-type="bibr" rid="CR26">26</xref>, <xref ref-type="bibr" rid="CR30">30</xref>, <xref ref-type="bibr" rid="CR31">31</xref>].</p><p>Additional non-RCT studies supporting a correlation between MIP and spirometry in patients with NMDs have also been performed in patients with Pompe disease, Guillain-Barré syndrome, and myasthenia gravis [<xref ref-type="bibr" rid="CR70">70</xref>, <xref ref-type="bibr" rid="CR74">74</xref>, <xref ref-type="bibr" rid="CR76">76</xref>, <xref ref-type="bibr" rid="CR77">77</xref>]. In a prospective cohort study in patients with Pompe disease, MIP and MEP were both strongly correlated with VC (<italic>r</italic> = 0.75 and <italic>r</italic> = 0.79, respectively) [<xref ref-type="bibr" rid="CR76">76</xref>]. Follow-up data (median 1.6 years) showed that VC (upright or supine) deteriorated by 0.9–1.2% points per year, respectively, with deteriorations in MIP and MEP of 3.2% (<italic>P</italic> = 0.018) and 3.8% (<italic>P</italic> < 0.01) per year, respectively [<xref ref-type="bibr" rid="CR76">76</xref>]. In a study of patients with respiratory muscle weakness and one study of multiple NMDs, MIP was found to significantly correlate with FRC (<italic>r</italic> = 0.62; <italic>P</italic> < 0.001) and VC (<italic>r</italic> = 0.88; <italic>P</italic> < 0.001) [<xref ref-type="bibr" rid="CR77">77</xref>]. A direct investigation of the sensitivity of MIP versus VC in patients with either Guillain-Barré syndrome (<italic>n</italic> = 40) or myasthenia gravis (<italic>n</italic> = 44) found a linear relationship between the 2 measurements [<xref ref-type="bibr" rid="CR70">70</xref>].</p><p>Taken together, these studies demonstrate that in patients with NMD there is a correlation between MIP and FVC, FEV<sub>1</sub>, body plethysmography, and diffusion techniques. In addition, MIP could have utility as a clinical endpoint in therapeutic trials for the treatment of neuromuscular diseases. A recent study in ALS patients with progressive respiratory dysfunction provided indirect evidence that reductions in MIP occur prior to reductions in FVC [<xref ref-type="bibr" rid="CR28">28</xref>]. In patients with ALS, clinicians detected the progression of respiratory dysfunction 6.5 months earlier when monitoring MIP compared with FVC alone. This indicates that MIP may be a more sensitive measure of respiratory disease progression, supporting its potential utility as a clinical trial endpoint.</p></sec><sec id="Sec8"><title>Relationship between MIP and nonpulmonary measures in patients with NMD</title><p>Walking tests are an integrated assessment of cardiac, pulmonary, circulatory, and muscular capacity, providing a measure of the functional exercise level required to undertake daily physical activities. Neuromuscular and pulmonary studies investigating the impact of MIP on ambulatory measures showed that, in some cases, improvements in MIP coincided with improvements in walking tests [<xref ref-type="bibr" rid="CR25">25</xref>, <xref ref-type="bibr" rid="CR34">34</xref>, <xref ref-type="bibr" rid="CR54">54</xref>, <xref ref-type="bibr" rid="CR74">74</xref>, <xref ref-type="bibr" rid="CR75">75</xref>, <xref ref-type="bibr" rid="CR78">78</xref>–<xref ref-type="bibr" rid="CR85">85</xref>]. Two studies were identified comparing MIP with walking tests: one in Pompe disease and one in ALS. The study of patients with Pompe disease [<xref ref-type="bibr" rid="CR74">74</xref>] receiving enzyme replacement therapy found that changes in the 6-min walk test (6MWT) were directionally consistent with changes in MIP. However, no such association was found in the study of patients with ALS [<xref ref-type="bibr" rid="CR75">75</xref>].</p></sec></sec><sec id="Sec9"><title>Discussion</title><p>Since the development of respiratory failure is a significant predictor of early death, many clinical trials currently employ established spirometry endpoints, including FVC, to evaluate an intervention in patients with NMD. However, as these endpoints are measures of overall pulmonary function, they may also be affected by factors that are independent of respiratory muscle dysfunction. Given that respiratory muscle dysfunction is common in neuromuscular diseases, directly evaluating diaphragm muscle strength by measuring MIP could complement spirometric endpoints in studies of patients with these diseases. In this regard, our analysis identified the use of MIP as an endpoint in several RCTs of pharmacologic therapies across a spectrum of diseases [<xref ref-type="bibr" rid="CR26">26</xref>, <xref ref-type="bibr" rid="CR30">30</xref>–<xref ref-type="bibr" rid="CR36">36</xref>]. These trials found MIP to be a clinically relevant outcome measure in chronic diseases when respiratory failure is secondary to respiratory muscle weakness.</p><p>However, diminished MIP does not always reliably confirm inspiratory muscle weakness. This is due to MIP measurement errors, including submaximal effort, poor transmission of intrathoracic pressure to the extrathoracic airways [<xref ref-type="bibr" rid="CR22">22</xref>] as well as NMD patient-device interface issues or additional chest wall alterations [<xref ref-type="bibr" rid="CR75">75</xref>]. For example, interface issues occur in patients with NMD with bulbar and/or facial weakness who have difficulty making a good lip seal. However, with proper training, MIP can be a reliable, accurate, and an early indicator of respiratory muscle weakness, which is more independent of existing lung abnormalities than FVC and VC [<xref ref-type="bibr" rid="CR22">22</xref>, <xref ref-type="bibr" rid="CR86">86</xref>–<xref ref-type="bibr" rid="CR88">88</xref>]. In this respect, a number of ongoing and planned clinical trials are evaluating MIP as a primary or secondary endpoint in studies of patients with NMDs (Table <xref rid="Tab2" ref-type="table">2</xref>).</p><p>In order to further validate MIP as a clinical endpoint in studies of patients with NMD, it is important to establish whether MIP is associated with clinically meaningful outcomes, such as time to ventilator support or even overall survival. In this regard, our analysis found that MIP was correlated with survival in patients with some NMDs (particularly ALS) and that MIP made an important contribution to predictive multivariate modeling analyses [<xref ref-type="bibr" rid="CR37">37</xref>–<xref ref-type="bibr" rid="CR41">41</xref>]. Studies in patients with ALS consistently found that higher MIP values were associated with increased survival. One study found MIP to be 100% sensitive as a threshold for predicting survival in patients with ALS at 18-months follow-up [<xref ref-type="bibr" rid="CR39">39</xref>]. Additionally, clinical studies that extended up to 5 years found a positive association between MIP and survival in a number of therapy areas [<xref ref-type="bibr" rid="CR41">41</xref>, <xref ref-type="bibr" rid="CR43">43</xref>].</p><p>Our analysis also suggests that MIP may be reflective of challenges faced by patients with NMD in their daily life. Two studies conducted in patients with NMD found that MIP correlated with improvements in QoL scores, including domains relating to sleep and fatigue [<xref ref-type="bibr" rid="CR18">18</xref>, <xref ref-type="bibr" rid="CR63">63</xref>]. Another study in patients with Pompe disease indicated that MIP was significantly correlated with the 6MWT [<xref ref-type="bibr" rid="CR74">74</xref>, <xref ref-type="bibr" rid="CR85">85</xref>]. These findings suggest that MIP may have useful long-term clinical relevance in patients with NMDs. Furthermore, in the majority of NMD studies examined, there was a strong correlation between MIP with other pulmonary measures, including with FVC, FEV<sub>1</sub>, VC, and TLC [<xref ref-type="bibr" rid="CR37">37</xref>, <xref ref-type="bibr" rid="CR72">72</xref>, <xref ref-type="bibr" rid="CR74">74</xref>–<xref ref-type="bibr" rid="CR76">76</xref>]. In addition, there is some evidence to indicate that, when respiratory muscle function starts to deteriorate, MIP measurements may decrease earlier than other pulmonary measures, suggesting that MIP may be a more sensitive method for monitoring patients [<xref ref-type="bibr" rid="CR28">28</xref>]. In ALS patients, for example, reduced inspiratory muscle strength (MIP) was noted after cessation of inspiratory muscle training despite other pulmonary measures remaining unchanged [<xref ref-type="bibr" rid="CR75">75</xref>].</p><p>In addition to MIP, the SNIP test can be used to measure respiratory muscle strength in the clinic. Like MIP, SNIP also reflects esophageal pressure in most patients, but a minority of patients will obtain substantially larger values on one test than the other [<xref ref-type="bibr" rid="CR89">89</xref>]. SNIP is measured by plugging one nostril while the other is free; this test is useful when there is facial weakness or dental malocclusion, which may make the MIP test difficult [<xref ref-type="bibr" rid="CR5">5</xref>]. It is a reproducible and accurate measure of inspiratory muscle strength [<xref ref-type="bibr" rid="CR90">90</xref>] and can be reliably performed and used even in infants and children [<xref ref-type="bibr" rid="CR91">91</xref>]. SNIP has been used to monitor respiratory muscle strength in patients with NMD [<xref ref-type="bibr" rid="CR92">92</xref>, <xref ref-type="bibr" rid="CR93">93</xref>]. However, it has limitations such as underestimating esophageal pressure swing in patients with severe nasal obstruction or airway diseases such as COPD, which impair pressure transmission [<xref ref-type="bibr" rid="CR94">94</xref>–<xref ref-type="bibr" rid="CR96">96</xref>]. Additionally, SNIP measures a fast contraction of inspiratory muscles, whereas MIP measures a sustained isometric contraction [<xref ref-type="bibr" rid="CR95">95</xref>]. As isometric muscle force of limb muscles is a standard measure of muscle function, it has been argued that in some NMDs, such as ALS, MIP might provide a more meaningful measurement of respiratory muscle function than SNIP [<xref ref-type="bibr" rid="CR95">95</xref>].</p><p>Our analysis of MIP as a clinical outcome measure in patients with NMDs is limited by the small number of published studies and small sample sizes. However, the fact that there are currently 12 registered trials in patients with NMD where MIP is being used as a clinical endpoint suggests that specific measurements of ventilatory function are important outcomes in these patient cohorts. These trials will provide further evidence of the reliability and utility of MIP. Additionally, MIP may prove to be more sensitive than FVC for assessing ventilatory dysfunction since spirometric pulmonary function tests are influenced by other factors (including scoliosis and other lung diseases), which may affect the reliability of results [<xref ref-type="bibr" rid="CR5">5</xref>, <xref ref-type="bibr" rid="CR68">68</xref>–<xref ref-type="bibr" rid="CR70">70</xref>].</p></sec><sec id="Sec10"><title>Conclusions</title><p>In summary, our analysis supports the use of MIP as a diagnostic of respiratory muscle dysfunction in patients with chronic NMDs and its utility as an endpoint in future clinical trials which monitor the efficacy of therapeutics in neuromuscular diseases. Through continued investigation of MIP in NMD, clinicians and researchers will gain a comprehensive understanding of the role of this direct measure of respiratory muscle strength in clinical practice.</p></sec></body><back><glossary><title>Abbreviations</title><def-list><def-item><term>6MWT</term><def><p>6-minute walk test</p></def></def-item><def-item><term>ALS</term><def><p>Amyotrophic lateral sclerosis</p></def></def-item><def-item><term>COPD</term><def><p>Chronic obstructive pulmonary disease</p></def></def-item><def-item><term>ESS</term><def><p>Epworth sleepiness scale</p></def></def-item><def-item><term>FEV<sub>1</sub></term><def><p>Forced expiratory volume in 1 second</p></def></def-item><def-item><term>FRC</term><def><p>Functional residual capacity</p></def></def-item><def-item><term>FVC</term><def><p>Forced vital capacity</p></def></def-item><def-item><term>MEP</term><def><p>Maximal expiratory pressure</p></def></def-item><def-item><term>MFI</term><def><p>Multidimensional fatigue inventory</p></def></def-item><def-item><term>MIP</term><def><p>Maximal inspiratory pressure</p></def></def-item><def-item><term>NMD</term><def><p>Neuromuscular disease</p></def></def-item><def-item><term>QoL</term><def><p>Quality of life</p></def></def-item><def-item><term>RCT</term><def><p>Randomized controlled trial</p></def></def-item><def-item><term>SF-36</term><def><p>36-Item short form</p></def></def-item><def-item><term>SNIP</term><def><p>Sniff nasal inspiratory pressure</p></def></def-item><def-item><term>TLC</term><def><p>Total lung capacity</p></def></def-item><def-item><term>VC</term><def><p>Vital capacity</p></def></def-item></def-list></glossary><ack><p>The authors wish to acknowledge Roger Hill PhD and Ryan Woodrow for medical writing and Dena McWain for editorial assistance in the preparation of the manuscript (Ashfield Healthcare Communications, Middletown, CT, USA).</p><sec id="FPar1"><title>Funding</title><p>BioMarin Pharmaceutical Inc. provided funding for this analysis and for medical writing and editorial support during manuscript development.</p></sec><sec id="FPar2"><title>Availability of data and materials</title><p>Not applicable.</p></sec><sec id="FPar3"><title>Authors’ contributions</title><p>All authors (BS EF TH DH JK SM DO MP MR HT PY) contributed to writing, reviewing, and analyzing the MIP review manuscript. All authors read and approved the final manuscript.</p></sec><sec id="FPar4"><title>Competing interests</title><p>BS received speaker honoraria from and is member of advisory boards for Audentes Inc, BioMarin Pharmaceutical Inc., and Genzyme, a Sanofi company.</p><p>MIP’s contribution to this project was supported by the NIHR Respiratory Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London who partially fund his salary. The views expressed in this publication are those of the authors and not necessarily those of the NHS, The National Institute for Health Research, or the Department of Health. He discloses receiving personal and institutional support for research and consultancy from BioMarin Pharmaceutical Inc. His institution has received research support from Genzyme.</p><p>PY received speaker honoraria from and is member of advisory boards for BioMarin Pharmaceutical Inc. and Genzyme, a Sanofi company.</p><p>EF received speaker honoraria from and/or is a member of advisory boards for BioMarin Pharmaceutical Inc. and Gilead Sciences Inc.</p><p>The other authors declare that they have no competing interests.</p></sec><sec id="FPar5"><title>Consent for publication</title><p>Not applicable.</p></sec><sec id="FPar6"><title>Ethics approval and consent to participate</title><p>Not applicable.</p></sec></ack><ref-list id="Bib1"><title>References</title><ref id="CR1"><label>1.</label><element-citation publication-type="journal"><person-group 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