RT Journal Article
SR Electronic
T1 Predictive factors of survival in amyotrophic lateral sclerosis patients with respiratory dysfunction
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P3521
VO 40
IS Suppl 56
A1 Inês Neves
A1 Hans Dabo
A1 Inês Belchior
A1 Anabela Marinho
A1 Tiago Pinto
A1 Miguel Gonçalves
A1 João Carlos Winck
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P3521.abstract
AB 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±768.0 L and 22.0±40.8 months after diagnosis. Mean duration of NIV was 19.6±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 ±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.