Abstract: Objective To reveal the clinical features of respiratory failure (RF) in dermatomyositis (DM) patients with interstitial lung disease (ILD), and to explore risk factors of RF in these patients. Methods The medical data of 122 DM patients with ILD were retrospectively reviewed: 40 developed RF (RF group), 82 did not develope RF (Non RF group). Clinical, laboratory and radiological variables were compared between RF patients and Non RF patients. Multivariate Logistic regression was used to analyze risk factors of RF. Results In RF patients, the female-male ratio was 3∶1, the median age at DM onset was 49.5 （42.3-58.6) years-old. There were 67.5%, 85.0% and 95.0% patients developed RF within 6 months, 1 year and 2 years after the onset of DM. The factors significantly associated with RF included DM onset age, clinically amyopathic dermatomyositis (CADM), pneumomediastinum (PNM), aspartate aminotransferase(AST), lactate dehydrogenase (LDH), albumin, neutrophil-lymphocyte ratio, platelet-lymphocyte ratio, anti-Jo-1 antibody presence and ground-glass opacities sign (POR)=1.791, P=0.025〕, higher AST level (OR=1.937, P=0.048), CADM diagnosis (OR=3.881, P=0.007) and ground-glass opacities sign (OR=4.187, P=0.014) were independent risk factors of RF in DM patients with ILD. ConclusionsRF occurs more often within 2 years of DM onset. The DM patients with older DM onset age, elevated AST level, CADM diagnosis or ground-glass opacities sign took higher risks for RF development.