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引用次数: 0
摘要
斯约格伦病(SjD)是一种全身性自身免疫性疾病,其特征是几乎所有患者都会出现三联主要症状(唾液和泪液干燥、疼痛和疲劳),1 至 2/3 的患者会出现腺外系统受累。在过去的十年中,有关流行病学、分类标准、系统活动评估和患者症状的知识不断增长。此外,随着对 SjD 病理生理学认识的不断深入,治疗方法也更具针对性。目前,SjD 的治疗以 EULAR 治疗指南为基础。但是,自提出这些建议以来,针对该病特定病理生理途径(主要是 B 淋巴细胞活化)的新药已在二期试验中显示出疗效。在本综述中,我们将总结系统疗法的现有证据,包括:1:1.结果评估方面的进展;2.目前有关靶向疾病修饰疗法和主要针对 B 淋巴细胞的生物药物的证据;3.正在进行的试验中的有前途药物概述。
[Sjögren's disease: From pathophysiology to treatment].
Sjögren's disease (SjD) is a systemic autoimmune disorder characterized by a triad of key symptoms affecting almost all patients (salivary and lacrimal dryness, pain and fatigue) and extra-glandular systemic involvement affecting one to two-thirds of patients. Over the past decade, knowledge of the epidemiology, classification criteria, assessment of systemic activity and symptoms presented by patients has grown. In addition, advances in understanding the pathophysiology of SjD have enabled a more targeted therapeutic approach. Current management of SjD is based on EULAR treatment guidelines. But since these recommendations, new drugs targeting specific pathophysiological pathways of the disease, and essentially B lymphocyte activation, have shown efficacy in phase 2 trials. In this review, we will summarize the available evidence on systemic therapies, including: 1. advances in outcome assessment, 2. current evidence on targeted disease-modifying therapies and biologic drugs targeting primarily B lymphocytes, 3. an overview of promising drugs being tested in ongoing trials.