Serum YKL-40 level is associated with severity of interstitial lung disease and poor prognosis in dermatomyositis with anti-MDA5 antibody

  • Lili Jiang
  • Youlian Wang
  • Qinglin Peng
  • Xiaoming Shu
  • Guochun WangEmail author
  • Xiaomu WuEmail author
Original Article



We aimed to investigate the clinical value of checking serum chitinase-3-like-1 protein (YKL-40) levels in anti-MDA5 antibody-positive dermatomyositis (anti-MDA5+DM) patients.


One hundred and five consecutive anti-MDA5+DM patients and 44 healthy controls were enrolled in this study. Baseline and follow-up serum YKL-40 were detected by ELISA. We evaluated the association of YKL-40 with rapidly progressive interstitial lung disease (RPILD), severity of interstitial lung disease (ILD), and ILD-related survival.


Forty-one out of 105 anti-MDA5+DM patients had RPILD at the time of serum sample collection (39.0%). Serum YKL-40 levels were significantly higher in anti-MDA5+DM patients with RPILD compared with those without (p = 0.011). One month after treatment, patients with aggravated ILD had increased YKL-40 levels, while those with stable/improved ILD had decreased YKL-40 levels. Higher serum levels of ferritin and YKL-40, as well as lower peripheral CD3+T cell counts, were independently associated with poorer prognosis. Kaplan–Meier survival curve showed that the 6 months survival rate in patients with high serum YKL-40 level (> 80 ng/ml) was significantly lower than that in patients with low YKL-40 level (≤ 80 ng/ml) (67% vs 89%, p < 0.01).


YKL-40 can be useful as an indicator for the occurrence of RPILD and correlates with severity of ILD and poor prognosis in anti-MDA5+DM patients. Closely monitoring and intensive treatment are suggested in anti-MDA5+DM patients showing high level of YKL-40, especially levels > 80 ng/ml.


Anti-melanoma differentiation-associated protein 5 antibody Dermatomyositis Rapidly progressive interstitial lung disease (RPILD) YKL-40 



The authors thank Xin Lu, He Chen, Lin Liang, Yamei Zhang, Lifang Ye, Yawen Shen, Hongxia Yang, and Hanbo Yang from the Department of Rheumatology, China-Japan Friendship Hospital, Beijing, China, for their assistance.

Funding information

This work was supported by Beijing Municipal Science and Technology Commission (Z181100001718063), the National Natural Science Foundation of China (81571603, 81701615), and the Capital Foundation of Medical Developments (2016-2-4063).

Compliance with ethical standards

The authors declare that manuscript has been read and approved by all authors. We have obtained the approval from the Research Review Committee (RRC) and the Ethical Review Committee (ERC) of the China-Japan Friendship Hospital. All the samples were obtained after the patients had provided written informed consent. This study was approved by the Research Review Committee and the Ethical Review Committee of the China-Japan Friendship Hospital (approval number 2016–117).




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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of MedicineNanchang UniversityNanchangChina
  2. 2.Department of RheumatologyJiangxi Provincial People’s hospitalNanchangChina
  3. 3.Department of RheumatologyChina-Japan Friendship HospitalBeijingChina
  4. 4.Department of NeurologyJiangxi Provincial People’s HospitalNanchangChina

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