This study was undertaken to investigate whether circulating lung biomarkers in idiopathic lung fibrosis and interstitial lung diseases correlated with connective tissue diseases. Between January 1960 and February 2019, researchers conducted a systematic review and meta-analysis of literature in MEDLINE and Embase databases. A total of 70 studies were enrolled in the review and 20 studies could be included in the meta-analysis. This study demonstrates that Interstitial lung disease (ILD) correlated with connective tissue diseases (CTD-ILD) and idiopathic pulmonary fibrosis (IPF) share common biomarkers, suggesting common pathophysiological pathways. The results suggested that disease-specific biomarkers are lacking and large longitudinal studies are required before the translational utility of the potential biomarkers in clinical practice. With the current availability of new effective therapies in ILDs, future research should evaluate response to treatment.