The present systematic review examined the effectiveness of bilevel noninvasive positive pressure ventilation (NIPPV) in the management of chronic respiratory failure (CRF) due to severe stable chronic obstructive pulmonary disease (COPD).Randomised controlled trials (RCTs) and non-RCTs (crossover design) of adults with severe stable COPD and CRF receiving bilevel NIPPV via nasal, oronasal or total face mask were identified from electronic databases and manual screening of journals and reference lists.Respiratory function (gas exchange, lung function, ventilatory/breathing pattern, respiratory muscle function and work of breathing) and health-related outcomes (dyspnoea, functional status, exercise tolerance, health-related quality of life (HRQOL), morbidity and mortality) were assessed.In total, 15 studies met the inclusion criteria: six RCTs and nine non-RCTs. RCTs did not find improved gas exchange with bilevel NIPPV, while non-RCTs did. Lung hyperinflation and diaphragmatic work of breathing were reduced in a nonrandomised subset. HRQOL and dyspnoea, the least studied outcomes, showed improvement with bilevel NIPPV.In a subset of individuals on maximal medical treatment regimes for severe stable chronic obstructive pulmonary disease, bilevel noninvasive positive pressure ventilation may have an adjunctive role in the management of chronic respiratory failure through attenuation of compromised respiratory function and improvement in health-related outcomes.KEYWORDS: Chronic obstructive pulmonary disease, chronic respiratory failure, noninvasive positive pressure ventilation, systematic review C hronic respiratory failure (CRF) due to chronic obstructive pulmonary disease (COPD) contributes a significant social and economic burden to individuals, families and the healthcare system. The incidence of COPD, in terms of its combined mortality and disability, was the 12th highest for diseases worldwide in 1990 and is expected to become the fifth highest by 2020, with mortality expected to increase fivefold by 2015 [1][2][3]. The rate of progression, the extent of airflow obstruction and airway hyperreactivity, and the impairment in alveolar ventilation and gas exchange contribute to the heterogeneity of COPD and the extent of chronic bronchitic versus emphysematous change that occurs. Reduced alveolar ventilation in CRF due to COPD results in nocturnal and daytime gas exchange abnormalities, sleep-disordered breathing, dyspnoea and increased work of breathing, which in turn lead to significant functional impairment, morbidity and mortality [4]. The eventual development of CRF is characterised by varying degrees of ventilation-perfusion mismatch, hypoxia and hypercapnia [5]. Reduced respiratory reserve associated with ongoing morbidity renders COPD patients at risk of acute respiratory decompensation [6,7]. Symptom management and prevention of respiratory decompensation are important in reducing morbidity and mortality associated with COPD.The use of bilevel noninvasive positive pressure ventilation (NIPPV)...