Variation in non-invasive ventilation use in amyotrophic lateral sclerosis

Objective: We sought to examine prevalence and predictors of non-invasive ventilation (NIV) in a composite cohort of amyotrophic lateral sclerosis (ALS) patients followed in a clinical trials setting (PRO-ACT database). Methods: NIV initiation and status were ascertained from response to question 12 of the revised ALS functional rating scale (ALSFRS-R). Factors affecting NIV use in patients with forced vital capacity (FVC) ≤ 50% of predicted were examined. Predictors of NIV were evaluated by Cox proportional hazard models and generalized linear mixed models. Results: Among 1,784 patients with 8,417 simultaneous ALSFRS-R and FVC% measures, NIV was used by 604 (33.9%). Of 918 encounters when FVC% ≤ 50%, NIV was reported in 482 (52.5%). Independent predictors of NIV initiation were lower FVC% (hazard ratio HR 1.27, 95% CI: 1.17-1.37 for 10% drop), dyspnea (HR 2.62, 95% CI: 1.87-3.69), orthopnea (HR 4.09, 95% CI: 3.02-5.55), lower bulbar and gross motor subscores of ALSFRS-R (HRs 1.09 (95% ...