Objective Obstructive sleep apnea (OSA) are increasingly recognized as important features in diffuse parenchymal lung diseases (DPLDs) with differed prevalence and impact reported. The aim of this study is to systematically review the prevalence of comorbid OSA and characterize its impact on clinical and outcome measurements in adults with DPLDs. Methods Publications addressing the prevalence of OSA in DPLDs and its impacts on DPLDs were selected from electronic databases. A random-effect model was used to estimate the pooled prevalence of OSA. Odds ratios (ORs) or mean differences (MDs) were used to assess the associations of OSA with clinical and outcome measurements. Heterogeneity was quantified by I.sup.2 with 95% confidence interval (95% CI). Results 4 studies comprising 643 participants were included. Overall, the pooled prevalence of OSA among DPLDs was 72% (95% CI: 65-79%; I.sup.2 = 75.4%). Moderate-severe OSA was observed in 40% patients (95% CI: 28-52%; I.sup.2 = 90.8%). The prevalence was higher as 76% in idiopathic pulmonary fibrosis (IPF) patients than in connective tissue associated-ILD or sarcoidosis (60%). Although oxygen desaturation during sleep was greater in OSA group compared with non-OSA patients, there was no difference in lung function or systematic comorbidities between the two groups. The associations between OSA and the mortality or disease progression of DPLDs were also systematically reviewed. Conclusion In conclusion, OSA is a common comorbidity in DPLD patients, affecting approximately three in four patients, which may exacerbate the nocturnal desaturation and have negative influence on the outcomes. Larger studies with more homogeneous samples are warranted.