Abstract
BackgroundAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, which rapidly leads to chronic respiratory failure requiring mechanical ventilation. Currently, forced vital capacity (FVC) < 50% is considered as physiologic marker for admitting patients to Noninvasive Positive Pressure Ventilation (NPPV) intervention, although it has been recently shown the median survival of patients with baseline FVC < 75% much shorter than median survival of patients with baseline FVC > 75%, independently by any treatment.AimTo assess the role of NPPV in improving outcome of ALS, a retrospective analysis was performed to investigate 1 year survival of ALS patients with FVC < 75% and nocturnal respiratory insufficiency, treated with NPPV, compared to a well-matched population of ALS patients, who refused or was intolerant to NPPV.MethodsWe investigated seventy-two consecutive ALS patients who underwent pulmonary function test. Forty-four presented a FVC > 75% and served as control group. Twenty-eight patients presented a FVC < 75% and showed, at polysomnography analysis, nocturnal respiratory insufficiency, requiring NPPV; sixteen were treated with NPPV, while twelve refused or were intolerant.ResultsIncreased survival rate at 1 year in patients with FVC < 75% treated with NPPV, as compared to those who refused or could not tolerate NPPV (p = 0.02), was observed. The median rate of decline in FVC% was slower in NPPV patients than in patients who did not use NPPV (95% CI: 0.72 to 1.85; p < 0.0001).ConclusionThis report demonstrates that early treatment with NPPV prolongs survival and reduces decline of FVC% in ALS.
Highlights
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive neuromuscular atrophy with early involvement of respiratory system, rapidly leading to pulmonary collapse, which requires mechanical ventilation and represents the major cause of mortality [1]
1 year survival rates showed a statistical significant difference between ALS patients with forced vital capacity (FVC) < 75% treated with Noninvasive Positive Pressure Ventilation (NPPV) and ALS patients with FVC < 75% who refused or were intolerant to NPPV (12/16 vs. 4/12; χ2 = 5.32; p = 0.02), while not statistical significant difference was found between patients with FVC > 75% vs. patients with FVC < 75% treated with NPPV (37/44 vs 12/16; χ2 = 0.408; p = 0.5)
Among survivors patients with FVC < 75%, the median rate FVC decline following initiation of NPPV was slower in patients who tolerated NPPV than in patients who did not tolerate NPPV, (Figure 2), FVC% slope change per month was (1.52 ± 0.3) in group 2 and 2.81 ± 0.8 in group 3 (p < 0.0001)
Summary
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive neuromuscular atrophy with early involvement of respiratory system, rapidly leading to pulmonary collapse, which requires mechanical ventilation and represents the major cause of mortality [1]. Forced vital capacity (FVC) is considered as physiologic marker to admit ALS patients to NPPV treatment [6]. Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, which rapidly leads to chronic respiratory failure requiring mechanical ventilation. Forced vital capacity (FVC) < 50% is considered as physiologic marker for admitting patients to Noninvasive Positive Pressure Ventilation (NPPV) intervention, it has been recently shown the median survival of patients with baseline FVC < 75% much shorter than median survival of patients with baseline FVC > 75%, independently by any treatment
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