Introduction: We performed a cross-sectional study to investigate the clinical usefulness of YKL-40 in patients with dermatomyositis (DM) and conducted a systematic review to summarize the clinical value of YKL-40 in patients with polymyositis (PM)/DM. Materials and methods: A cross-sectional study and a systematic review were performed to study the clinical value of YKL-40 in patients with PM/DM. In the cross-sectional study, a total of 65 DM patients with mean age 47.71 years and 30 healthy controls with mean age 47.73 years were included. Serum YKL-40 level was detected using enzyme-linked immunosorbent assay, and its association with clinical and laboratory parameters was analyzed by Spearman correlation analysis. The diagnostic value of serum YKL-40 in patients with DM was assessed by receiver operating characteristic (ROC) curve analysis. In the systematic review, electronic databases of OVID Embase, OVID Medline, and web of science were searched to collect studies that reported clinical use of YKL-40 in patients with PM/DM.Results: In the cross-sectional study, serum YKL-40 level was higher in patients with DM than in healthy controls (median [interquartile range]: 84.09 [52.72-176.4] ng/mL vs 27. 37 [12.30-53.58] ng/mL, p<0.0001). Serum levels of YKL-40 were associated with the course of DM (r=-0.469, p<0.001), CRP (r=0.303, p=0.043), CK (r=0.263, p=0.037), and global disease activity (r=0.628, p<0.001). The area under the ROC curve was 0.835 (95% con dence interval 0.751-0.920). In the systematic review, a total of 4 studies were included with moderate to high quality. Serum level of YKL-40 has the possibility for diagnosing PM/DM, identifying PM/DM patients with interstitial lung disease (ILD) or rapid progress ILD, and predicting death.Conclusion: Serum YKL-40 level is a possible useful biomarker for PM/DM diagnosis and may be used to predict prognosis.