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

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 componen...

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Bibliographic Details
Published in:Lupus 2017-12, Vol.26 (14), p.1550-1555
Main Authors: Hristova, M H, Stoyanova, V S
Format: Article
Language:English
Subjects:
Antigen-antibody complexes
Apoptosis
Apoptosis - immunology
Autoantibodies
Autoantibodies - immunology
Complement activation
Complement System Proteins - immunology
Glomerulonephritis
Humans
IgA nephropathy
Immune System - immunology
Immunoglobulin A
Immunoglobulins
Lupus
Lupus Erythematosus, Systemic - genetics
Lupus Erythematosus, Systemic - immunology
Lupus Erythematosus, Systemic - physiopathology
Lupus nephritis
Lupus Nephritis - immunology
Lupus Nephritis - physiopathology
Nephritis
Pathogenesis
Systemic lupus erythematosus
Thrombosis
Thyroiditis
Vasculitis
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Summary: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.
ISSN:0961-2033
1477-0962
DOI:10.1177/0961203317709347