Autoantibodies against complement components in systemic lupus erythematosus – role in the pathogenesis and clinical manifestations

Abstract

Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common. These antibodies cause conformational changes leading to pathological activation or inhibition of complement with organ damage and/or limited capacity of the immune system to clear immune complexes and apoptotic debris. Finally, we summarize the role of complement antibodies in the pathogenesis of systemic lupus erythematosus and discuss the mechanism of some related clinical conditions such as infections, thyroiditis, thrombosis, acquired von Willebrand disease, etc.

Analysis of multiple organ damage and clinical immunological characteristics in systemic lupus erythematosus patients with hematologic involvement

“…Compared with SLE patients without hematologic involvement, the levels of complement C3 and C4 in SLE patients with hematologic involvement were lower. Previous studies 12 , 13 have shown that IgG, C3 and C4 can be used as indicators to monitor the active stage of SLE disease, and hematologic involvement in the clinical diagnostic guidelines of SLE disease was also classified as the performance of SLE disease activity. The results of this study were proved to be reliable.…”

Sir William Osler's fatal trip to Scotland: “Mrs M” and the University Grants Committee

“…However, the association of acquired angioedema with SLE is welldescribed, and some cases have been attributed to decreased C1-INH levels caused by increased catabolism or autoantibodies. 47,48 Autoantibodies against complement components in patients with SLE can also cause hypocomplementemic urticarial vasculitis, 49,50 a distinctive type of vasculitis with multi-organ involvement, which could explain "all sorts of skin lesions" and "chronic nephritis" in the case of Mrs M. (Current opinion supports Osler's statement that "there is really no warrant for separating too sharply angio-neurotic oedema and urticaria." 36 ) The association of angioedema with HSP is less prominent, although both conditions were present in one of Osler's cases 41 :…”

Renal function protection and the mechanism of ginsenosides: Current progress and future perspectives

“…LN pathogenesis mainly includes immune complex lesions ( Anders and Fogo, 2014 ) and epigenetic abnormalities ( Dieker, et al 2007 ). In addition, abnormalities in the complement system ( Hristova and Stoyanova, 2017 ), sexual hormone disorders ( Feng, et al 2010 ), and environmental effects are also related to the occurrence and development of LN.…”