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Updated: Feb 5, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Diagnostic value of KL-6 in idiopathic interstitial pneumonia
Peiyan Zheng1, Xiaoqing Liu1, Huimin Huang1
1Department of Allergy and Clinical Immunology, Guangzhou Institute of Respiratory Health, State Key Laboratory of Respiratory Disease, National Clinical Research Center for Respiratory Disease, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou 510120, China.
Background:
Idiopathic interstitial pneumonia (IIP) can induce type II alveolar epithelial cell proliferation and pulmonary basement membrane damage and subsequent release of Krebs von den Lungen-6 antigen (KL-6) to the bloodstream. This study investigated the diagnostic and prognostic value of serum KL-6 levels for IIP.
Methods:
One hundred five patients with lung disease were divided into IIP (n=75) and non-IIP groups (n=30) according to pathological and computed tomography findings. Serum KL-6 levels were evaluated in blood samples from all subjects. Nineteen IIP group patients were also subjected to a longitudinal study of disease progression and serum KL-6 levels over time.
Results:
Serum KL-6 levels were significantly higher in the IIP group vs. the non-IIP group [1,096.0 (565.0-1,544.0) vs. 226.0 (173.5-346.5) U/mL; P<0.01]. Within the IIP group, serum KL-6 levels differed significantly between patients with and without concomitant disease or pulmonary infection (Z=-2.475, P=0.013). In a receiver operating characteristic (ROC) curve analysis, the area below the curve for serum KL-6 was 0.911 [95% confidence interval (CI): 0.847-0.975, P<0.001], indicating a good diagnostic performance for IIP, with a cut-off level of 485 U/mL, sensitivity of 85.33%, specificity of 90.00%, positive predictive value (PPV) of 95.52%, negative predictive value (NPV) of 71.05%, and Kappa value of 0.70. Accordingly, the serum KL-6 and clinical diagnostic results were consistent. Moreover, in the longitudinal study, the serum KL-6 levels differed significantly from before to after treatment in patients with exacerbated or improved disease (P=0.004 and P=0.043, respectively), whereas no obvious changes were observed in patients with stable disease (P=0.692).
Conclusions:
The serum KL-6 level is a valuable and significant diagnostic marker of IIP and a useful predictor of clinical prognosis.
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