Revisiting the complement system in systemic lupus erythematosus.

Revisiting the complement system in systemic lupus erythematosus. Expert Rev Clin Immunol. 2020 Mar 31;: Authors: Sharma M, Vignesh P, Tiewsoh K, Rawat A Abstract Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organ systems, characterized by the production of autoantibodies. Numerous mechanisms contribute to the pathogenesis and development of autoantibodies 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 forms of lupus in individuals with monogenic defects in any one of the early complement components such as C1q, C1s, C1r, C2, or C4.Areas covered: 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 searched and retrieved from PubMed for this purpose.Expert opinion: Complements have a dual role in the pathogenesis of SLE. On the one hand, deficiency of the complement components predisposes to lupus, while on the other hand, excess complement activation plays a ...
Source: Expert Review of Clinical Immunology - Category: Allergy & Immunology Tags: Expert Rev Clin Immunol Source Type: research