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Progranulin correlated with rapid progressive interstitial lung disease in dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody

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Abstract

Objectives

This study aimed to detect the expression of progranulin (PGRN) and elucidate associations with clinical features in dermatomyositis (DM) patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody.

Methods

We enrolled 40 DM patients with anti-MDA5 antibody, 20 patients with antisynthetase syndrome (ASS; disease control), and 20 healthy individuals (healthy control, HC). The clinical features of patients with anti-MDA5 antibody and anti-histidyl-tRNA antibody were collected. The level of PGRN in the serum was tested by ELISA.

Results

The PGRN levels in DM patients with anti-MDA5 antibody (166.74 ± 97.95 ng/ml) were significantly higher than those in patients with ASS (82.66 ± 40.50 ng/ml; p < 0.001) and in HC (42.34 ± 18.69 ng/ml; p < 0.001). Patients with rapid progressive interstitial lung disease (RP-ILD) in DM with anti-MDA5 antibody (213.57 ± 114.05 ng/ml) had higher levels of PGRN than those without RP-ILD (135.51 ± 72.41 ng/ml; p = 0.012). ROC analysis showed an AUC value at 0.715 (95% CI, 0.541–0.888) for diagnosis of RP-ILD in DM patients with anti-MDA5 antibody. The expression of PGRN was positively correlated with the levels of ALT, AST, CK, LDH and ferritin (all p < 0.05).

Conclusions

Our results indicated PGRN had great potential as a valuable serum marker of RP-ILD in DM with anti-MDA5 antibody.

Key Points

The level of PGRN was elevated in DM patients with anti-MDA5 antibody, especially for those with RP-ILD.

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Data availability

All data included in our study will be shared as anonymized data via request from any qualified investigator.

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Funding

The work was supported by a grant from the Science and Technology Commission Foundation of Beijing (Z191100006619012).

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Authors and Affiliations

  1. Department of Rheumatology, China-Japan Friendship Hospital, No.2 Yinghua East st, Chaoyang District, Beijing, China

    Li Shanshan, Zhang Yamei, Lu Xin & Wang Guochun

  2. Department of Radiology, China-Japan Friendship Hospital, Beijing, China

    Zhang Ling

Authors
  1. Li Shanshan
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  2. Zhang Yamei
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  3. Zhang Ling
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  4. Lu Xin
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  5. Wang Guochun
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Contributions

LS collected clinical data, laboratory data, and wrote the manuscript. ZY finished the experiment. ZL collected chest CT images. LX and WG supervised the overall study and critically revised the manuscript.

Corresponding author

Correspondence to Wang Guochun.

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Ethics approval

The study was approved by the ethics committee of the China-Japan Friendship Hospital. All persons gave their informed consent prior to participation in the study.

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Shanshan, L., Yamei, Z., Ling, Z. et al. Progranulin correlated with rapid progressive interstitial lung disease in dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody. Clin Rheumatol 41, 757–763 (2022). https://doi.org/10.1007/s10067-021-05816-2

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  • DOI: https://doi.org/10.1007/s10067-021-05816-2

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