Department of Rheumatology and Immunology, Peking University People's Hospital, 11 Xizhimen South Street, Beijing, 100044, China.
Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China.
Arthritis Res Ther. 2023 Jun 15;25(1):104. doi: 10.1186/s13075-023-03070-2.
Adult-onset Still's disease (AOSD) is a systemic autoinflammatory disorder of unknown etiology. B cells are critical participants in different rheumatic diseases, and their roles in AOSD are rarely investigated. This study aimed to unveil the B cell subset features in AOSD and provide evidence for B cell-based diagnosis and targeted therapies of AOSD.
B cell subsets in the peripheral blood of AOSD patients and healthy controls (HCs) were detected by flow cytometry. Firstly, the frequencies of B cell subsets were compared. Then, the correlation analysis was performed to explore the correlation between B cell subsets and clinical manifestations in AOSD. Finally, unbiased hierarchical clustering was performed to divide AOSD patients into three groups with different B cell subset features, and the clinical characteristics of the three groups were compared.
The frequencies of B cell subsets were altered in AOSD patients. Disease-promoting subsets (such as naïve B cells, double negative B cells (DN B cells), and plasmablasts) increased, and potential regulatory subsets (such as unswitched memory B cells (UM B cells) and CD24CD27 B cells (B10 cells)) decreased in the peripheral blood of AOSD patients. In addition, the altered B cell subsets in AOSD correlated with the clinical and immunological features, such as immune cells, coagulation features, and liver enzymes. Intriguingly, AOSD patients could be divided into three groups with distinct B cell immunophenotyping: group 1 (naïve B cells-dominant), group 2 (CD27 memory B cells-dominant), and group 3 (precursors of autoantibody-producing plasma cells-dominant). Moreover, these three group patients demonstrated differential manifestations, including immune cells, liver or myocardial enzymes, coagulation features, and systemic score.
B cell subsets are significantly altered in AOSD patients, potentially contributing to the disease pathogenesis. These findings would inspire B cell-based diagnosis and targeted therapies for this refractory disease.
成人斯蒂尔病（AOSD）是一种病因不明的系统性自身炎症性疾病。B 细胞是不同风湿性疾病的关键参与者，但其在 AOSD 中的作用很少被研究。本研究旨在揭示 AOSD 中 B 细胞亚群的特征，并为基于 B 细胞的 AOSD 诊断和靶向治疗提供证据。
采用流式细胞术检测 AOSD 患者和健康对照（HC）外周血中的 B 细胞亚群。首先，比较 B 细胞亚群的频率。然后，进行相关性分析，以探讨 AOSD 中 B 细胞亚群与临床表现之间的相关性。最后，进行无偏层次聚类，将 AOSD 患者分为具有不同 B 细胞亚群特征的三组，并比较三组的临床特征。
AOSD 患者的 B 细胞亚群频率发生改变。促病亚群（如幼稚 B 细胞、双阴性 B 细胞（DN B 细胞）和浆母细胞）增加，潜在调节亚群（如未转换记忆 B 细胞（UM B 细胞）和 CD24CD27 B 细胞（B10 细胞））减少。此外，AOSD 中改变的 B 细胞亚群与临床和免疫学特征相关，如免疫细胞、凝血特征和肝酶。有趣的是，AOSD 患者可分为具有不同 B 细胞免疫表型的三组：组 1（幼稚 B 细胞占主导）、组 2（CD27 记忆 B 细胞占主导）和组 3（自身抗体产生浆母细胞前体细胞占主导）。此外，这三组患者表现出不同的表现，包括免疫细胞、肝或心肌酶、凝血特征和全身评分。
AOSD 患者的 B 细胞亚群显著改变，可能有助于疾病的发病机制。这些发现将激发基于 B 细胞的诊断和靶向治疗这种难治性疾病。
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