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Serum KL-6 is associated with the severity of interstitial lung disease in Chinese patients with polymyositis and dermatomyositis

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Abstract

Objective

This cross-sectional study was designed to assess the clinical significance of the serum KL-6 in the diagnosis of interstitial lung disease (ILD) in patients with idiopathic inflammatory myopathy (IIM).

Methods

We measured serum KL-6 levels in 184 patients with IIM using a chemiluminescent enzyme immunoassay and compared KL-6 levels between patients with and without ILD, according to other clinical features.

Results

IIM patients with ILD had significantly higher serum KL-6 levels than those without ILD (776.5 [372.3–1378.8] vs. 297.5 [204.75–599.3] U/ml, P < 0.001). At a cut-off of 461.5 U/ml identified by ROC curve, serum KL-6 yielded a sensitivity of 70.2% and specificity of 73.9% for ILD in IIM patients. IIM patients with an elevated serum KL-6 were more likely to have clinical symptoms of mechanic’s hands (P = 0.002), anti-Jo-1 antibody positivity (P = 0.021), dysphagia (P = 0.039), hoarseness (P < 0.001), and polyarthritis/polyarthralgia (P < 0.001). Significant inverse correlations were found between serum KL-6 levels and pulmonary function tests (P < 0.01), including forced vital capacity (FVC, %Pred), total lung capacity (TLC, %Pred), and diffusing capacity for carbon monoxide (DLCO, %Pred).

Conclusions

Serum KL-6 offers high sensitivity and specificity for the diagnosis of IIM-associated ILD and is inversely correlated with pulmonary function deterioration. Serum KL-6 may represent a promising biomarker for monitoring ILD severity in IIM patients.

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Funding

This study was supported by the National Natural Science Foundation of China (81771780, 81471615, 81102268, 81302610), the Outstanding Youth Fund of Peking Union Medical College Hospital (JQ201504), the CAMS Initiative for Innovative Medicine (2017-I2M-3-001), and the 2016 PUMCH Science Fund for Junior faculty (pumch-2016-1.5).

Author information

Author notes

  1. Chaojun Hu and Chanyuan Wu contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College &Chinese Academy of Medical Sciences, No.1 Shuaifuyuan, Beijing, 100730, China

    Chaojun Hu, Chanyuan Wu, Enhao Yang, Dong Xu, Yong Hou, Jiuliang Zhao, Mengtao Li, Xiaofeng Zeng & Qian Wang

  2. Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, No.1 Shuaifuyuan, Beijing, 100730, China

    Chaojun Hu, Chanyuan Wu, Enhao Yang, Dong Xu, Yong Hou, Jiuliang Zhao, Mengtao Li, Xiaofeng Zeng & Qian Wang

  3. Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College &Chinese Academy of Medical Sciences, No.1 Shuaifuyuan, Beijing, 100730, China

    Hui Huang & Zuojun Xu

Authors
  1. Chaojun Hu
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  2. Chanyuan Wu
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  3. Enhao Yang
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  4. Hui Huang
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  5. Dong Xu
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  6. Yong Hou
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  7. Jiuliang Zhao
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  8. Mengtao Li
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  9. Zuojun Xu
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  10. Xiaofeng Zeng
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  11. Qian Wang
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Corresponding authors

Correspondence to Xiaofeng Zeng or Qian Wang.

Ethics declarations

This study was approved by the Medical Ethics Committee of Peking Union Medical College Hospital (S-478). The methods were carried out in accordance with the principles stated in the Declaration of Helsinki, and informed consent was obtained from each patient.

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Hu, C., Wu, C., Yang, E. et al. Serum KL-6 is associated with the severity of interstitial lung disease in Chinese patients with polymyositis and dermatomyositis. Clin Rheumatol 38, 2181–2187 (2019). https://doi.org/10.1007/s10067-019-04501-9

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  • DOI: https://doi.org/10.1007/s10067-019-04501-9

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