Clinical stratification of 1318 Primary Sjögren's Syndrome patients

• Published in: Seminars in arthritis and rheumatism 2024-10, Vol.68, p.152537, Article 152537
• Main Authors: Fang, Jinxia, Wang, Jiajia, Luo, Jing, Wang, Ping, Zhang, Jin, Chen, Dan, Ye, Wenjing, Zhang, Yi, Pan, Shaobiao, Wang, Xiaobing
• Format: Article
• Language: English
• Subjects: Clinical stratification; Minor salivary gland biopsy; Primary Sjögren's Syndrome; RNA sequencing
• ISSN: 0049-0172; 1532-866X; 1532-866X
• DOI: 10.1016/j.semarthrit.2024.152537

•This study offers an in-depth analysis of the clinical characteristics observed in a large cohort of Chinese patients with Primary Sjögren's Syndrome (pSS).•We employed unsupervised clustering based on distinct systemic involvement to categorize patients into four unique subgroups.•These subgroups demonstrate significant differences in their clinical manifestations, laboratory findings, and transcriptomic profiles, underscoring the heterogeneity within pSS.•Our innovative classification approach not only provides a novel perspective on pSS but also enhances understanding of the complex immune mechanisms underlying the disease. 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 identifi