title: Initiation of non-invasive ventilation in amyotrophic lateral sclerosis and clinical practice guidelines: Single-centre, retrospective, descriptive study in a national reference centre.
abstract: In amyotrophic lateral sclerosis (ALS), respiratory muscle weakness leads to respiratory failure. Non-invasive ventilation (NIV) maintains adequate ventilation in ALS patients. NIV alleviates symptoms and improves survival. In 2006, French guidelines established criteria for NIV initiation based on limited evidence. Their impact on clinical practice remains unknown. Our objective was to describe NIV initiation practices of the main French ALS tertiary referral centre with respect to guidelines. In this retrospective descriptive study, 624 patients followed in a single national reference centre began NIV between 2005 and 2013. We analysed criteria used to initiate NIV, including symptoms, PaCO2, forced vital capacity, maximal inspiratory pressures and time spent with SpO2<90% at night. At NIV initiation, 90% of patients were symptomatic. Median PaCO2was 48 mmHg. The main criterion to initiate NIV was 'symptoms' followed by 'hypercapnia' in 42% and 34% of cases, respectively. NIV was initiated on functional parameters in only 5% of cases. Guidelines were followed in 81% of cases. In conclusion, despite compliance with French guidelines, the majority of patients are treated at the stage of symptomatic daytime hypoventilation, which suggests that NIV is initiated late in the course of ALS. Whether this practice could be improved by changing guidelines or increasing respiratory-dedicated resources remains to be determined.
authors.affiliation: a Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique , France.|b AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale (Département '3S') , Paris , France.|c Centre Hospitalier Universitaire Dijon Bourgogne , Service de Pneumologie et Soins Intensifs Respiratoires , Dijon , France.
authors.forename: Marjolaine
authors.initials: M
authors.lastname: Georges
authors.affiliation: d AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Biostatistiques, Paris , France.
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Golmard
authors.affiliation: b AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale (Département '3S') , Paris , France.
authors.forename: Claudia
authors.initials: C
authors.lastname: Llontop
authors.affiliation: b AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale (Département '3S') , Paris , France.|e An Shams University , Cairo , Egypt , and.
authors.forename: Amr
authors.initials: A
authors.lastname: Shoukri
authors.affiliation: f AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Neurologie , Paris , France.
authors.forename: François
authors.initials: F
authors.lastname: Salachas
authors.affiliation: a Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique , France.|b AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale (Département '3S') , Paris , France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Similowski
authors.affiliation: a Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique , France.|b AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale (Département '3S') , Paris , France.
authors.forename: Capucine
authors.initials: C
authors.lastname: Morelot-Panzini
authors.affiliation: a Sorbonne Universités, UPMC Univ Paris 06, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique , France.|b AP-HP , Groupe Hospitalier Pitié-Salpêtrière Charles Foix, Service de Pneumologie et Réanimation Médicale (Département '3S') , Paris , France.
authors.forename: Jésus
authors.initials: J
authors.lastname: Gonzalez-Bermejo
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000628:therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation / Q000379:methods*; D017410:Practice Guidelines as Topic; D012016:Reference Values; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000628:therapy*; D012189:Retrospective Studies
keywords: Non-invasive ventilation; amyotrophic lateral sclerosis; guidelines evaluation; respiratory muscles