ABSTRACTBACKGROUND:Respiratory failure is the most common cause of death in patients with
amyotrophic lateral sclerosis (ALS), and morbidity is related to poor
quality of life (QOL). Non-invasive ventilation (NIV) may be associated with
prolonged survival and QOL in patients with ALS.OBJECTIVES:To assess whether NIV is effective and safe for patients with ALS in terms of
survival and QOL, alerting the health system.DESIGN AND SETTING:Systematic review was conducted in accordance with Preferred Reporting Items
for Systematic Reviews and Meta-Analyses reporting standards using
population, intervention, comparison, and outcome strategies.METHODS:The Cochrane Library, CENTRAL, MEDLINE, LILACS, EMBASE, and CRD databases
were searched based on the eligibility criteria for all types of studies on
NIV use in patients with ALS published up to January 2022. Data were
extracted from the included studies, and the findings were presented using a
narrative synthesis.RESULTS:Of the 120 papers identified, only 14 were related to systematic reviews.
After thorough reading, only one meta-analysis was considered eligible. In
the second stage, 248 studies were included; however, only one systematic
review was included. The results demonstrated that NIV provided relief from
the symptoms of chronic hypoventilation, increased survival, and improved
QOL compared to standard care. These results varied according to clinical
phenotype.CONCLUSIONS:NIV in patients with ALS improves the outcome and can delay the indication
for tracheostomy, reducing expenditure on hospitalization and occupancy of
intensive care unit beds.SYSTEMATIC REVIEW REGISTRATION:PROSPERO database: CRD42021279910 —https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=279910.