Department of Oral & Maxillofacial Oncology and Surgery, Osaka University Graduate School of Dentistry, Osaka 565-0871, Japan.
Int J Mol Sci. 2023 Jul 6;24(13):11179. doi: 10.3390/ijms241311179.
Sjögren's syndrome (SS) is an autoimmune disease characterized by the involvement of exocrine glands such as the salivary and lacrimal glands. The minor salivary glands, from which tissue samples may be obtained, are important for the diagnosis, evaluation of therapeutic efficacy, and genetic analyses of SS. In the onset of SS, autoantigens derived from the salivary glands are recognized by antigen-presenting dendritic cells, leading to the activation of T and B cells, cytokine production, autoantibody production by plasma cells, the formation of ectopic germinal centers, and the destruction of salivary gland epithelial cells. A recent therapeutic approach with immune checkpoint inhibitors for malignant tumors enhances the anti-tumor activity of cytotoxic effector T cells, but also induces SS-like autoimmune disease as an adverse event. In the treatment of xerostomia, muscarinic agonists and salivary gland duct cleansing procedure, as well as sialendoscopy, are expected to ameliorate symptoms. Clinical trials on biological therapy to attenuate the hyperresponsiveness of B cells in SS patients with systemic organ involvement have progressed. The efficacy of treatment with mesenchymal stem cells and chimeric antigen receptor T cells for SS has also been investigated. In this review, we will provide an overview of the pathogenesis of salivary gland lesions and recent trends in therapeutic approaches for SS.
干燥综合征（SS）是一种自身免疫性疾病，其特征是外分泌腺（如唾液腺和泪腺）受累。从小唾液腺中可以获得组织样本，对于 SS 的诊断、疗效评估和遗传分析非常重要。在 SS 的发病过程中，来自唾液腺的自身抗原被抗原呈递树突状细胞识别，导致 T 和 B 细胞激活、细胞因子产生、浆细胞产生自身抗体、异位生发中心形成以及唾液腺上皮细胞破坏。最近，免疫检查点抑制剂治疗恶性肿瘤增强了细胞毒性效应 T 细胞的抗肿瘤活性，但也会引发 SS 样自身免疫性疾病等不良事件。在治疗口干症方面，拟副交感神经药和唾液腺导管清洁术以及唾液腺内镜检查有望改善症状。针对全身性器官受累的 SS 患者 B 细胞高反应性的生物治疗临床试验也在进展中。间充质干细胞和嵌合抗原受体 T 细胞治疗 SS 的疗效也在研究中。在这篇综述中，我们将概述唾液腺损伤的发病机制和 SS 的治疗方法的最新趋势。
