Department of Rheumatology and Immunology, Taizhou Hospital of Zhejiang Province affiliated to Wenzhou Medical University, Taizhou, Zhejiang, China.
Department of Ultrasonography, The First Affiliated Hospital, Wenzhou Medical University, Wenzhou, Zhejiang, China.
Semin Arthritis Rheum. 2024 Oct;68:152537. doi: 10.1016/j.semarthrit.2024.152537. Epub 2024 Aug 10.
Primary Sjögren's Syndrome (pSS) is a complex autoimmune disorder characterized by diverse clinical manifestations yet lacking effective therapeutic strategies currently. This study aims to gain a thorough understanding of the clinical landscape of pSS and further delineate its clinical subtypes, thereby enabling the efficient management for pSS.
We conducted a cross-sectional observational study of 1318 pSS patients. The pSS patients were categorized and compared based on gender, anti-SSA antibodies, and labial salivary gland biopsies (LGSB). Unsupervised clustering analysis was employed to identify pSS subtypes using systemic involvement among patients. Furthermore, we assessed clinical and biological variances among these subtypes.
Through group comparisons, we observed more pronounced extraglandular manifestations among male patients, SSA-negative group, and those with positive LGSB results. Based on systemic involvement, pSS patients were categorized into four groups. C1 exhibited minimal systemic involvement, lacking hematologic or serologic manifestations, with the lowest ESSDAI scores. C2 presented with serologic changes in all patients, partial joint involvement, and no hematologic systemic manifestations. C3 lacked joint involvement but all members displayed hematologic systemic involvement, with higher rates of renal, cutaneous, and systemic manifestations. C4 encompassed patients with joint and hematologic involvement, displaying the highest ESSDAI scores. The positivity rates of antibodies, immunological parameters, and inflammatory markers exhibited significant differences among the groups. Furthermore, notable variances were observed in the expression of peripheral blood transcriptomic modules among these groups.
In this cohort study, we summarized the clinical characteristics of Chinese patients with pSS and identified four distinct subgroups of pSS based on systemic involvement, revealing clinical and molecular disparities that unveil distinct pathobiological endotypes. Our findings hold significant implications for clinical management.
原发性干燥综合征（pSS）是一种复杂的自身免疫性疾病，具有多种临床表现，但目前缺乏有效的治疗策略。本研究旨在全面了解 pSS 的临床特征，并进一步描绘其临床亚型，从而为 pSS 的有效管理提供依据。
我们对 1318 例 pSS 患者进行了横断面观察性研究。根据性别、抗 SSA 抗体和唇腺活检（LGSB）对 pSS 患者进行分类和比较。采用无监督聚类分析对患者的系统受累情况进行分析，以识别 pSS 亚型。此外，我们评估了这些亚型之间的临床和生物学差异。
通过组间比较，我们发现男性患者、抗 SSA 抗体阴性组和 LGSB 阳性组的外分泌腺表现更为明显。根据系统受累情况，将 pSS 患者分为 4 组。C1 组患者仅有轻微的系统受累，无血液学或血清学表现，ESSDAI 评分最低。C2 组患者所有患者均存在血清学改变，部分关节受累，无血液系统表现。C3 组无关节受累，但所有患者均有血液系统受累，肾脏、皮肤和全身表现的发生率较高。C4 组患者存在关节和血液系统受累，ESSDAI 评分最高。各组间抗体、免疫参数和炎症标志物的阳性率存在显著差异。此外，这些组之间外周血转录组模块的表达也存在明显差异。
在这项队列研究中，我们总结了中国 pSS 患者的临床特征，并根据系统受累情况确定了四个不同的 pSS 亚组，揭示了临床和分子差异，揭示了不同的病理生物学表型。我们的研究结果对临床管理具有重要意义。
