Sjögren syndrome or Sjögren disease is a chronic form of autoimmune epithelitis characterized by lymphocytic infiltration of the exocrine glands, particularly the salivary and lacrimal glands, leading to progressive glandular dysfunction and subsequent xerostomia and xerophthalmia. Other common manifestations include pain and fatigue, various systemic manifestations and non-Hodgkin’s lymphoma. Sjögren syndrome is therefore a complex and disabling disease associated with a reduced quality of life and with considerable long-term damage. Most of the available treatments are merely symptomatic with limited efficacy in both preventing glandular damage and suppressing systemic disease activity. In the past 10 years, great progress has been made in understanding the pathophysiology of Sjögren syndrome, opening new avenues towards a more targeted and individualized therapeutic approach to the disease. Indeed, several randomized controlled trials have just been completed or are poised to commence evaluating the effectiveness of novel drugs targeting both innate and adaptive immune pathways, including pro-inflammatory cytokines, the type I interferon system, B cell activation, B cell and T cell co-stimulation pathway, and ectopic germinal centre formation. Novel clinical trials are also ongoing exploring various targeted approaches (that is, IgG recycling inhibition, nuclease therapy and CAR-T cell therapy) for Sjögren syndrome.

干燥综合征或干燥病是一种慢性形式的自身免疫性上皮炎，其特征是外分泌腺，特别是唾液腺和泪腺的淋巴细胞浸润，导致进行性腺功能障碍以及随后的口干症和干眼症。其他常见表现包括疼痛和疲劳、各种全身表现和非霍奇金淋巴瘤。因此，干燥综合征是一种复杂的致残性疾病，与生活质量下降和相当大的长期损害相关。大多数可用的治疗方法仅仅是对症治疗，在预防腺体损伤和抑制全身性疾病活动方面的功效有限。在过去的十年中，对干燥综合征病理生理学的理解取得了巨大进展，为对该疾病采取更有针对性和个体化的治疗方法开辟了新途径。事实上，一些随机对照试验刚刚完成或准备开始评估针对先天性和适应性免疫途径的新药的有效性，包括促炎细胞因子、I 型干扰素系统、B 细胞激活、B 细胞和 T 细胞共刺激途径和异位生发中心形成。新的临床试验也在不断探索针对干燥综合征的各种靶向方法（即 IgG 回收抑制、核酸酶治疗和 CAR-T 细胞治疗）。