Background: Respiratory insufficiency is the most common cause of mortality among patients with a neuromuscular disease. Methods: We explored the effects of high frequency wall compression and intrapulmonary percussive ventilation, compared with standard care or no treatment, on the lung volume and capacity, and quality of life in patients with neuromuscular disease during respiratory infections or in stable periods. We further assessed the effects of these two interventions on clinical value, complications, and survival. The literature search was performed on Embase, MEDLINE, CINAHL, CENTRAL and PEDro. Randomised controlled trials and cross-over studies were eligible. Results: Five studies were included, and results were presented narratively. High frequency wall compression was not shown to be superior to standard care in terms of lung volume and capacity, quality of life, complications, and survival rate. Compared with standard care, intrapulmonary percussive ventilation showed non-significant differences in terms of lung volume and capacity, and the risk of respiratory infection. Standard care was nevertheless associated with a significantly higher risk of days of hospitalisation (Incidence Rate Ratio 8.5 [1.1-67]) and of antibiotic use than intrapulmonary percussive ventilation (Incidence Rate Ratio 43 [6-333]). Conclusions: Due to large variety of reported outcomes, missing data and limited number of studies, no meta-analysis could be conducted. The results should be interpreted with caution as the results have a very low certainty of evidence and reported outcomes have a high risk of bias. The evidence for high frequency wall compression and intrapulmonary percussive ventilation is still insufficient to draw final conclusions. Protocol registration: PROSPERO ID: CRD42017064703.

Background: Respiratory insufficiency is the most common cause of mortality among patients with a neuromuscular disease. Methods: We followed the PRISMA statement for systematic reviews. We explored the effects of high frequency wall compression and intrapulmonary percussive ventilation, compared to a control intervention, on the lung volume and capacity, and quality of life in patients with neuromuscular disease. We further assessed the effects of these two interventions on clinical value, complications, and survival. The literature search was performed on 30/06/2020 in Embase, MEDLINE, CENTRAL, PEDro and CINAHL on 6/07/2020. Inclusion criteria: patients with neuromuscular disease; interventions of interest mentioned above; randomised controlled trials comparing these interventions with a control intervention. Results: Five studies were included, and results were presented narratively. High frequency wall compression was not shown to be superior to standard care in terms of lung volume and capacity, quality of life, complications, and survival rate. Compared with standard care, intrapulmonary percussive ventilation showed non-significant differences in terms of lung volume and capacity, and the risk of respiratory infection. Standard care was nevertheless associated with a significantly higher risk of days of hospitalisation (Incidence Rate Ratio 8.5 [1.1-67]) and of antibiotic use than intrapulmonary percussive ventilation (Incidence Rate Ratio 43 [6-333]). The assessment with the risk of bias tool 2.0 showed a high risk of bias for all outcomes. Moreover, the evidence is of very low-quality for all outcomes. Conclusions: Due to large variety of reported outcomes, missing data and limited number of studies, no meta-analysis could be conducted. The results should be interpreted with caution as the results have a very low certainty of evidence and reported outcomes have a high risk of bias. The evidence for high frequency wall compression and intrapulmonary percussive ventilation is still insufficient to draw final conclusions. Registration: PROSPERO ID: CRD42017064703.