Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital (PUMCH); Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China.
Chin Med J (Engl). 2023 Oct 5;136(19):2297-2306. doi: 10.1097/CM9.0000000000002539.
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease with high prevalence and possible poor prognosis. Though the pathogenesis of pSS has not been fully elucidated, B cell hyperactivity is considered as one of the fundamental abnormalities in pSS patients. It has long been identified that Janus kinases-signal transducer and activator of transcription (JAK-STAT) signaling pathway contributes to rheumatoid arthritis and systemic lupus erythematosus. Recently, increasing numbers of studies have provided evidence that JAK-STAT pathway also has an important role in the pathogenesis of pSS via direct or indirect activation of B cells. Signal transducer and activator of transcription 1 (STAT1), STAT3, and STAT5 activated by various cytokines and ribonucleic acid contribute to pSS development, respectively or synergically. These results reveal the potential application of Janus kinase inhibitors for treatment of pSS, which may fundamentally improve the quality of life and prognosis of patients with pSS.
原发性干燥综合征（pSS）是一种系统性自身免疫性疾病，具有较高的患病率和可能较差的预后。尽管 pSS 的发病机制尚未完全阐明，但 B 细胞过度活跃被认为是 pSS 患者的基本异常之一。长期以来，人们已经认识到 Janus 激酶-信号转导和转录激活因子（JAK-STAT）信号通路有助于类风湿关节炎和系统性红斑狼疮的发生。最近，越来越多的研究提供了证据表明，JAK-STAT 通路通过直接或间接激活 B 细胞，在 pSS 的发病机制中也具有重要作用。各种细胞因子和核糖核酸激活的信号转导和转录激活因子 1（STAT1）、STAT3 和 STAT5 分别或协同促进 pSS 的发展。这些结果揭示了 Janus 激酶抑制剂治疗 pSS 的潜力，这可能从根本上改善 pSS 患者的生活质量和预后。
