Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Campania L. Vanvitelli, Naples, Italy.
Centre for Rheumatology, Department of Medicine, University College London, UK.
Clin Exp Rheumatol. 2019 May-Jun;37 Suppl 118(3):167-174. Epub 2019 Apr 26.
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterised by xerostomia and xerophthalmia. In at least one-third of patients, the disease may be complicated by extraglandular involvement. Due to the lack of evidence-based recommendations, current therapeutic options for pSS are mainly empirical, often reflecting their use in other autoimmune diseases. Nevertheless, recent advances in the understanding of pathogenic pathways in pSS encourage the belief that blocking them may be of value in the treatment of the disease. Despite failing to demonstrate efficacy in clinical trials, because of the well-established role of B-lymphocytes in the pathogenesis of pSS, rituximab has been the most frequently used to date, but with much less success than in the treatment of patients with rheumatoid arthritis, vasculitis and lupus. However, in the last few years a number of other biologics have been developed and are under investigation. The aim of this article is to review the use of biologic therapies in pSS.
原发性干燥综合征（pSS）是一种以口干和眼干为特征的系统性自身免疫性疾病。至少有三分之一的患者可能会出现腺体外表现。由于缺乏循证推荐，目前 pSS 的治疗选择主要是经验性的，通常反映了它们在其他自身免疫性疾病中的应用。然而，近年来对 pSS 发病机制中致病途径的认识的进展，使人相信阻断这些途径可能对治疗这种疾病有价值。尽管在临床试验中未能证明其疗效，但由于 B 淋巴细胞在 pSS 发病机制中的作用已得到充分证实，利妥昔单抗是迄今为止应用最广泛的药物，但疗效远不如在治疗类风湿关节炎、血管炎和狼疮患者时那样显著。然而，在过去几年中，已经开发出了许多其他生物制剂，并正在进行研究。本文的目的是综述生物疗法在 pSS 中的应用。
