Ritter Jacob, Chen Yidan, Stefanski Ana-Luisa, Dörner Thomas
Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany; Berlin Institute of Health (BIH), Berlin, Germany.
Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany; German Rheumatism Research Center (DRFZ), a Leibniz Gesellschaft, Berlin, Germany.
Joint Bone Spine. 2022 Nov;89(6):105406. doi: 10.1016/j.jbspin.2022.105406. Epub 2022 May 7.
Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease characterized by sicca symptoms, systemic manifestations and constitutional symptoms substantially diminishing patient's quality of life. In this review, we summarize recent recommendations for management of pSS patients and current clinical studies in pSS addressing unmet medical needs. Expanding knowledge about disease pathogenesis and the introduction of validated outcome measures, such as capturing disease activity (ESSDAI) and patient-reported outcomes (ESSPRI) have shaped recent developments. In contrast, lack of evidence for current treatment options remarkably limits the management of pSS patients as reflected by the 2019 updated EULAR recommendations for management of Sjögren's syndrome. In this context, symptomatic treatment is usually appropriate for sicca symptoms, whereas systemic treatment is reserved for moderate to severe organ manifestations including care by a multidisciplinary team in centers of expertise. Most promising targets for new treatment modalities are based on immunopathological insights and include direct B cell targeting strategies, targeting co-stimulation by CD40/CD40L blocking, inhibition of key cytokine activity (BLyS/BAFF, type I interferon) and intracellular signaling pathways.
原发性干燥综合征（pSS）是一种慢性自身免疫性疾病，其特征为干燥症状、全身表现和全身症状，严重降低患者的生活质量。在本综述中，我们总结了近期关于pSS患者管理的建议以及针对未满足医疗需求的pSS当前临床研究。对疾病发病机制的认识不断扩展以及引入经过验证的结局指标，如捕捉疾病活动（欧洲抗风湿病联盟干燥综合征疾病活动指数[ESSDAI]）和患者报告结局（欧洲抗风湿病联盟干燥综合征患者报告指数[ESSPRI]），塑造了近期的发展。相比之下，正如2019年更新的欧洲抗风湿病联盟干燥综合征管理建议所反映的那样，目前治疗选择缺乏证据显著限制了pSS患者的管理。在这种情况下，对症治疗通常适用于干燥症状，而全身治疗则用于中度至重度器官表现，包括在专业中心由多学科团队进行护理。新治疗模式最有前景的靶点基于免疫病理学见解，包括直接靶向B细胞策略、通过阻断CD40/CD40L靶向共刺激、抑制关键细胞因子活性（B淋巴细胞刺激因子/增殖诱导配体[BLyS/BAFF]、I型干扰素）和细胞内信号通路。
