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Clinical and immunological characteristics and prognosis of patients with autoantibody negative dermatomyositis: a case control study

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Abstract

Objectives

Myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinctive dermatomyositis (DM) clinical phenotypes. The aim of this study is to explicate the clinical and immunological features of MSAs-negative DM patients.

Methods

A total of 515 individuals diagnosed with DM was screened from 2013 to 2022 and 220 DM patients were enrolled in this retrospective cohort. Clinical and laboratory data of these patients were analyzed.

Results

MSAs-negative DM patients were categorized into two groups: MAAs-negative (MSAs (−)/MAAs (−)) group and MAAs-positive (MSAs (−)/MAAs (+)) group. The percentage of Raynaud’s phenomenon (P=0.026) was higher in the MSAs (−)/MAAs (+) DM patients than the MSAs-positive DM patients and MSAs (−)/MAAs (−) DM patients. The proportion of rapidly progressive interstitial lung disease (RP-ILD) in the MSAs-negative DM patients was lower than that in the MSAs-positive group. The MSAs (−)/MAAs (+) group had a higher proportion of organizing pneumonia and usual interstitial pneumonia (P=0.011), and elevated eosinophils in their bronchoalveolar lavage fluid (P=0.008). Counts of lymphocytes (P=0.001) and CD16+CD56+ natural killer (NK) cells (P=0.012) were higher in the MSAs-negative group. Additionally, the percentage of CD4+TNFα+ (P=0.040), CD4+IFNγ+ (P=0.037), and CD4+IL-2+ (P=0.018) cells among total CD4+ T cells were higher in the MSA-negative DM patients compared with the MSAs-positive DM patients. Besides, MSAs-negative patients demonstrated a more favorable prognosis than MSAs-positive patients. Multivariable regression analysis identified advanced onset age, higher level of carcinoembryonic antigen (CEA), and RP-ILD as risk factors for mortality in DM patients.

Conclusions

Compared with MSAs-positive group, MSAs-negative DM patients suffered less from organ involvement compared with MSAs-positive group and tend to have better prognosis.

Key Points

MSAs-negative DM patients exhibited distinct characteristics in comparison with MSAs-positive DM patients:

  • The MSAs (−)/MAAs (+) DM patients demonstrated a higher prevalence of organizing pneumonia (OP) and usual interstitial pneumonia (UIP), and elevated eosinophil counts in bronchoalveolar lavage fluid.

  • CEA levels were lower in MSAs-negative patients compared with MSAs-positive patients.

  • Elevated counts of lymphocytes and CD16+CD56+ NK cells were identified in the MSAs-negative patients. Additionally, proportions of CD4+TNFα+, CD4+IFNγ+, and CD4+IL-2+ cells among total CD4+ T cells were higher in the MSAs-negative DM patients compared with DM MSAs-positive DM patients.

  • MSAs-negative DM patients had a more favorable prognosis than MSAs-positive DM patients. A multivariable regression analysis revealed the advanced onset age, high CEA levels, and RP-ILD were risk factors for mortality in DM patients.

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The original contributions to this research are included in the article; for further inquiries, please contact the corresponding author.

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Acknowledgements

The authors appreciate the assistance of Dr. Hui Zhi and Dr. Wentao Ni in verification of the image of HRCT.

Funding

This work was supported by the National Natural Science Foundation of China (82371804), Beijing Natural-Science Foundation (L222017), Peking University People’s Hospital Scientific Research Development Foundation (RDX2023-03). 

Author information

Author notes

  1. Xiaoyan Xing and Yuzhou Gan contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology and Immunology and Beijing Key Laboratory for Rheumatism and Immune Diagnosis (BZ0135), Peking University People’s Hospital, Beijing, 100044, China

    Xiaoyan Xing, Yuzhou Gan, Wanxing Mo, Jian Zhang, Naidi Wang, Kai Zhang, Yuhui Li & Jing He

  2. Center of Clinical Immunology, Peking University, Beijing, 100044, China

    Xiaoyan Xing, Yuzhou Gan, Wanxing Mo, Jian Zhang, Naidi Wang, Kai Zhang, Yuhui Li & Jing He

  3. Department of Rheumatology and Immunology, Peking University People’s Hospital, Qingdao, 266111, Shandong Province, China

    Ke Ma

  4. Department of Rheumatology, Hulunbeier People’s Hospital, Hulunbuir, 021008, Inner Mongolia, China

    Lihua Zhang

  5. Department of Rheumatology, Hebei Hospital of Traditional Chinese Medicine, Shijiazhuang, 050200, Hebei Province, China

    Lin Ma

  6. Institute of Systems Biomedicine, School of Basic Medical Sciences, Peking University Health Science Center, Beijing, 100191, China

    Dan Lu

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Contributions

XYX and YZG conceived and designed the study and wrote the manuscript. WXM revised the manuscript. JZ, NDW, FYZZ, KZ, KM, LHZ, LM, and DL collected the data. XYX and YZG analyzed the data. YHL and JH conceptualized, supervised, and edited the manuscript. All authors contributed to the article and approved the submitted version.

Corresponding authors

Correspondence to Yuhui Li or Jing He.

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Ethics

The study was approved by the ethics committee of Peking University People’s Hospital and the study complied with the Declaration of Helsinki guidelines (2020PHB114-01).

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Xing, X., Gan, Y., Mo, W. et al. Clinical and immunological characteristics and prognosis of patients with autoantibody negative dermatomyositis: a case control study. Clin Rheumatol 43, 1145–1154 (2024). https://doi.org/10.1007/s10067-024-06873-z

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  • DOI: https://doi.org/10.1007/s10067-024-06873-z

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