title: Factors predicting survival in amyotrophic lateral sclerosis patients on non-invasive ventilation.
abstract: Non invasive ventilation (NIV) improves quality of life and extends survival in amyotrophic lateral sclerosis (ALS) patients. However, few data exist about the factors related to survival. We intended to assess the predictive factors that influence survival in patients after NIV initiation.
  
  
  
  Patients who started NIV from 2000 to 2014 and were tolerant (compliance ≥ 4 hours) were included; demographic, disease related and respiratory variables at NIV initiation were analysed. Statistical analysis was performed using the Kaplan-Meier test and Cox proportional hazard models.
  
  
  
  213 patients were included with median survival from NIV initiation of 13.5 months. In univariate analysis, the identified risk factors for mortality were severity of bulbar involvement (HR 2), Forced Vital Capacity (FVC) % (HR 0.99) and ALSFRS-R (HR 0.97). Multivariate analysis showed that bulbar involvement (HR 1.92) and ALSFRS-R (HR 0.97) were independent predictive factors of survival in patients on NIV.
  
  
  
  In our study, the two prognostic factors in ALS patients following NIV were the severity of bulbar involvement and ALSFRS-R at the time on NIV initiation. A better assessment of bulbar involvement, including evaluation of the upper airway, and a careful titration on NIV are necessary to optimize treatment efficacy.
authors.affiliation: a Pulmonary Department, Hospital Universitari de Bellvitge, Barcelona, Spain .|b IDIBELL Instituto de Investigación Biomédica de Bellvitge, Barcelona, Spain .
authors.forename: Nuria
authors.initials: N
authors.lastname: Gonzalez Calzada
authors.affiliation: a Pulmonary Department, Hospital Universitari de Bellvitge, Barcelona, Spain .|b IDIBELL Instituto de Investigación Biomédica de Bellvitge, Barcelona, Spain .
authors.forename: Enric
authors.initials: E
authors.lastname: Prats Soro
authors.affiliation: c Rehabilitation Department , and.
authors.forename: Lluis
authors.initials: L
authors.lastname: Mateu Gomez
authors.affiliation: a Pulmonary Department, Hospital Universitari de Bellvitge, Barcelona, Spain .
authors.forename: Esther
authors.initials: E
authors.lastname: Giro Bulta
authors.affiliation: a Pulmonary Department, Hospital Universitari de Bellvitge, Barcelona, Spain .|b IDIBELL Instituto de Investigación Biomédica de Bellvitge, Barcelona, Spain .
authors.forename: Ana
authors.initials: A
authors.lastname: Cordoba Izquierdo
authors.affiliation: b IDIBELL Instituto de Investigación Biomédica de Bellvitge, Barcelona, Spain .|d Neurology Department , Hospital Universitari de Bellvitge , Barcelona , Spain.
authors.forename: Monica
authors.initials: M
authors.lastname: Povedano Panades
authors.affiliation: a Pulmonary Department, Hospital Universitari de Bellvitge, Barcelona, Spain .|b IDIBELL Instituto de Investigación Biomédica de Bellvitge, Barcelona, Spain .
authors.forename: Jordi
authors.initials: J
authors.lastname: Dorca Sargatal
authors.affiliation: a Pulmonary Department, Hospital Universitari de Bellvitge, Barcelona, Spain .|b IDIBELL Instituto de Investigación Biomédica de Bellvitge, Barcelona, Spain .
authors.forename: Eva
authors.initials: E
authors.lastname: Farrero Muñoz
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality* / Q000523:psychology / Q000628:therapy*; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation / Q000379:methods*; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D014797:Vital Capacity / Q000502:physiology
keywords: ALS; prognostic; survival