Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
Expert Rev Clin Immunol. 2020 Apr;16(4):397-408. doi: 10.1080/1744666X.2020.1745063. Epub 2020 Apr 6.
: Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease, characterized by the production of autoantibodies. Numerous mechanisms contribute to the pathogenesis and autoimmunity in SLE. One of the most important mechanisms is the defective function of the early complement components that are involved in clearing the immune-complexes and apoptotic debris. Major evidence supporting this hypothesis is the development of severe lupus in individuals with monogenic defects in any one of the early complement components such as , or .: In this review, we discuss hereditary defects in classical complement components and their clinical manifestations, acquired defects of complements in lupus, the role of complements in the pathogenesis of antiphospholipid antibody syndrome and lupus nephritis, and laboratory assessment of complement components and their functions. Articles from the last 20 years were retrieved from PubMed for this purpose.: Complements have a dual role in the pathogenesis of SLE. On one hand, deficiency of complement components predisposes to lupus, while, on the other, excess complement activation plays a role in the organ damage. Understanding the intricacies of the role of complements in SLE can pave way for the development of targeted therapies.
系统性红斑狼疮 (SLE) 是一种多系统自身免疫性疾病，其特征是产生自身抗体。许多机制导致 SLE 的发病机制和自身免疫。最重要的机制之一是早期补体成分的功能缺陷，这些成分参与清除免疫复合物和凋亡碎片。支持这一假说的主要证据是在任何一种早期补体成分（如 、 或 ）的单基因缺陷个体中出现严重狼疮。在这篇综述中，我们讨论了经典补体成分的遗传性缺陷及其临床表现、狼疮中补体的获得性缺陷、补体在抗磷脂抗体综合征和狼疮肾炎发病机制中的作用以及补体成分及其功能的实验室评估。为此目的，从 PubMed 中检索了过去 20 年的文章。补体在 SLE 的发病机制中具有双重作用。一方面，补体成分的缺乏使狼疮易感性增加，另一方面，补体的过度激活在器官损伤中起作用。了解补体在 SLE 中的作用的复杂性可以为开发靶向治疗铺平道路。
