@article {NevesP3521, author = {In{\^e}s Neves and Hans Dabo and In{\^e}s Belchior and Anabela Marinho and Tiago Pinto and Miguel Gon{\c c}alves and Jo{\~a}o Carlos Winck}, title = {Predictive factors of survival in amyotrophic lateral sclerosis patients with respiratory dysfunction}, volume = {40}, number = {Suppl 56}, elocation-id = {P3521}, year = {2012}, publisher = {European Respiratory Society}, abstract = {Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that causes severe respiratory dysfunction which is the major cause of death. Early management of respiratory symptoms may improve outcomes and survival.Aim: Describe survival of patients with ALS and respiratory dysfunction and identify predictive factors of survival.Methods: Retrospective analysis of patients with ALS evaluated in an outpatient setting. Ventilatory support data was screened. Kaplan-Meier survival analysis was performed and predictive factors were evaluated by Cox multivariate regression.Results: 60 patients (25 females) with a median age of 64.5 years (range 34-80) were analyzed. At presentation, 33 patients (55\%) had slow bulbar-onset and 27 (45\%) rapid bulbar-onset. Non-invasive ventilation (NIV) was initiated in 52 patients (86.7\%), with a mean vital capacity of 1698.0{\textpm}768.0 L and 22.0{\textpm}40.8 months after diagnosis. Mean duration of NIV was 19.6{\textpm}23.7 months. Mechanical assisted cough was used in 23 patients (38.3\%). Gastrostomy was performed in 21 patients (17 rapidly bulbar) and tracheostomy in 10 (9 rapidly bulbar) after a mean time of 13.6 {\textpm}17.0 months under NIV. The 5-year survival was 48\%. The median overall survival and survival after respiratory muscle aids initiation was significantly higher in slowly bulbar patients compared with rapidly bulbar (p=0.03 and p\<0.01, respectively). In multivariate analysis, predictive factors of survival were younger age, slow bulbar-onset, and early NIV initiation.Conclusion: Survival may be prolonged in patients with ALS and respiratory dysfunction with early NIV initiation. Age and bulbar onset have significant negative impact on survival.}, issn = {0903-1936}, URL = {https://erj.ersjournals.com/content/40/Suppl_56/P3521}, eprint = {https://erj.ersjournals.com/content/40/Suppl_56/P3521.full.pdf}, journal = {European Respiratory Journal} }