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Cluster analysis of clinical phenotypes in idiopathic inflammatory myopathy patients complicated with cardiac involvement

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Abstract

Objective

This study aimed to classify idiopathic inflammatory myopathy (IIM) patients with cardiac involvement (IIM-CI) into different categories based on their clinical phenotypes via cluster analysis and to explore their differences in outcomes.

Methods

IIM-CI patients admitted to Peking Union Medical College Hospital from January 2015 to June 2021 were retrieved. The clinical data, laboratory examinations, and treatment were retrospectively reviewed, and the outcome was traced. A second-order clustering method was employed for categorization.

Results

A total of 88 IIM-CI patients were enrolled in this study and were classified into two categories through cluster analysis. Category I consisted of patients who exhibited distinct cardiac structural and functional changes, such as enlargement of atriums and/or ventricles, along with the remarkable heart insufficiency biomarkers, whereas patients of category II displayed more widely systemic injuries and intensive skeletal muscle weakness. In comparison, pulmonary hypertension (58.8% vs 16.7%, p < 0.01), arrhythmia (82.4% vs 27.8%, p < 0.01), and positive serum anti-mitochondrial-M2 antibody (52.9% vs 5.6%, p < 0.01) were more prevalent in category I than in category II, and serum N-terminal pro-B-type natriuretic peptide levels (1703.5 pg/L vs 364.0 pg/L, p = 0.02) were significantly elevated in category I, whereas skeletal muscle weakness (50.0% vs 74.1%, p = 0.02), interstitial lung disease (20.6% vs 63.0%, p < 0.01), skin rash (11.8% vs 48.1%, p < 0.01), arthralgia (2.9% vs 27.8%, p < 0.01), fever (2.9% vs 27.8%, p < 0.01), and dysphagia (2.9% vs 22.2%, p < 0.01) were more common in category II patients. Heart failure was the primary cause of death in category I, but severe pneumonia was predominantly responsible for deaths in category II.

Conclusion

Two categories of IIM-CI were identified based on clinical features with distinctive characteristics. Two categories exhibited differences in clinical manifestations, autoantibody profiles, and the primary cause of death.

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Data availability

Data are available upon reasonable request by any qualified researchers who engage in rigorous, independent scientific research, and will be provided following review and approval of a research proposal and Statistical Analysis Plan (SAP) and execution of a Data Sharing Agreement (DSA). All data relevant to the study are included in the article.

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Acknowledgements

We wish to acknowledge all the patients who participated in our study and our cardiologist colleagues for their participation.

Funding

This research was supported by the National Natural Sciences Foundation of China (82071840, 82271856), CAMS Innovation Fund for Medical Sciences (CIFMS) (2020-I2M-C&T-B-013), and National High Level Hospital Clinical Research Funding (2022-PUMCH-C-037, 2022-PUMCH-A-108).

Author information

Author notes

  1. Jianling Dong, Xia Meng, and Haojie Xu contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No.1 Shuai-Fu-Yuan, Dongcheng District, Beijing, 100730, China

    Jianling Dong, Xia Meng, Haojie Xu, Huaxia Yang, Jiaxin Zhou & Lidan Zhao

  2. National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, 100730, China

    Jianling Dong, Xia Meng, Haojie Xu, Huaxia Yang, Jiaxin Zhou & Lidan Zhao

  3. Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China

    Jianling Dong, Xia Meng, Haojie Xu, Huaxia Yang, Jiaxin Zhou & Lidan Zhao

  4. Department of Rheumatology, Mianyang Central Hospital, Mianyang, 621000, China

    Jianling Dong & Jing Yang

Authors
  1. Jianling Dong
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  2. Xia Meng
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  5. Jing Yang
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  6. Jiaxin Zhou
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  7. Lidan Zhao
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Contributions

Conception: L.Z.; methodology: L.Z., J.D., H.X., X.M.; data curation: H.X., X.M., J.D., J.Z.; analysis: X.M., J.D., H.X., J.Y.; interpretation and writing: H.X., X.M., J.D.; supervision: J.Z., H.Y., L.Z. All authors were involved in drafting this paper or revising it critically for key intellectual content, and all authors approved this version to be published. Dr. J.Z. and Dr. L.Z. had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.

Corresponding authors

Correspondence to Jiaxin Zhou or Lidan Zhao.

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During the preparation of this work, the author(s) used ChatGPT 4.0 in order to polish the language of this article. After using this tool, the authors reviewed and edited the content as needed and take full responsibility for the content of the publication.

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Dong, J., Meng, X., Xu, H. et al. Cluster analysis of clinical phenotypes in idiopathic inflammatory myopathy patients complicated with cardiac involvement. Clin Rheumatol (2024). https://doi.org/10.1007/s10067-024-06986-5

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