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Phenotypes of patients with systemic sclerosis in the Chinese Han population: a cluster analysis

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Abstract

Objective

Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is commonly subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) based on the extent of skin involvement. This subclassification may not reflect the full range of clinical phenotypic variation. This study aimed to investigate clinical features and aggregation of patients with SSc in Chinese based on SSc manifestations and organ involvements, in order to achieve precise treatment of SSc early prevention of complications.

Methods

In total 287 SSc patients were included in this study. A cluster analysis was applied according to 13 clinical and serologic variables to determine subgroups of patients. Survival rates between obtained clusters and risk factors affecting prognosis were also compared.

Result

In this study, six clusters were observed: cluster 1 (n = 66) represented the skin type, with all patients showing skin thickening. In cluster 2 (n = 56), most patients had vascular and articular involvement. Cluster 3 (n = 14) individuals mostly had cardiac and pulmonary involvement. In cluster 4 (n = 52), the gastrointestinal type, 50 patients presented with stomach symptoms and 28 patients presented with esophageal symptoms. In cluster 5 (n = 50), patients barely had any major organ involvement. Cluster 6 (n = 49) included 46% of all patients presenting with renal crisis.

Conclusion

The results of our cluster analysis study implied that limiting SSc patient subgroups to those based only on skin involvement might not capture the full heterogeneity of the disease. Organ damage and antibody profiles should be considered when identifying homogeneous patient groups with a specific prognosis.

Key Points

• Provides a new method of categorizing SSc patients.

• Can better explain disease progression and guide subsequent treatment.

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Data availability

The datasets used and analyzed during the current study are available from the corresponding author on reasonable request.

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Funding

This work was supported by the National Natural Science Foundation of China (Youth fund project, Grant NO.82201994) and the Natural Science Foundation of Shandong Province (General Program, Grant NO. ZR2022MH016 and Youth fund project, Grant NO. ZR2021QH043).

Author information

Author notes

  1. Yaqi Zhao and Wei Xu contributed equally to this work.

Authors and Affiliations

  1. Department of Rheumatology and Immunology, Shandong Provincial Hospital, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, China

    Yaqi Zhao, Wei Xu, Zhenzhen Ma & Qingrui Yang

  2. Department of Rheumatology and Immunology, Shandong Provincial Hospital Affiliated to Shandong First Medical University, Jinan, Shandong, China

    Wei Xu, Xinya Li, Baocheng Liu, Suyan Yan, Zhenzhen Ma & Qingrui Yang

  3. Department of Rheumatology and Immunology, the Affiliated Hospital of Weifang Medical College, Weifang, Shandong, China

    Wenfeng Gao

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  1. Yaqi Zhao
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Contributions

Yaqi Zhao and WeiXu participated in the study design and literature review, performed statistical analysis and presentation of the results, and participated in the drafting and review of the manuscript. Yaqi Zhao, WeiXu, Wenfeng Gao, Xinya Li, Baocheng Liu, and Suyan Yan collected clinical data. Zhenzhen Ma and Qingrui Yang participated in the study design, drafting, and review of the manuscript. All authors contributed to the article and approved the submitted version.

Corresponding authors

Correspondence to Zhenzhen Ma or Qingrui Yang.

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Ethics approval

The study was conducted according to the guidelines of the Declaration of Helsinki and approved by the Ethics Committee of Shandong Provincial Hospital, Shandong First Medical University (NSFC:NO.2022–413). All participants signed the informed consent form.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Significance and innovations

1. Cluster analysis of systemic sclerosis patients has been studied internationally, but less so in the Chinese population. The present study shows how the subclassification of cases by subgroups (clusters) that consider organ damage, antibodies, and demographic data can better explain the trajectories of the disease and could have subsequent implications in terms of prognosis or treatments that can be better explained in future prospective studies.

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Zhao, Y., Xu, W., Gao, W. et al. Phenotypes of patients with systemic sclerosis in the Chinese Han population: a cluster analysis. Clin Rheumatol 43, 1635–1646 (2024). https://doi.org/10.1007/s10067-024-06936-1

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