The complement system I: innate defects of complement cascade proteins excluding hereditary angioedema
Authors:
Z. Chovancová; R. Hakl; J. Litzman
Authors‘ workplace:
Ústav klinické imunologie a alergologie, Fakultní nemocnice u sv. Anny v Brně, Lékařská fakulta Masarykovy univerzity, Brno
Published in:
Čes-slov Pediat 2021; 76 (4): 211-217.
Category:
Overview
The complement system consists of more than 50 soluble and membrane-bound proteins. It is a crucial part of the innate immune system. Individual components of the complement cascade are cleaved during the activation process into smaller fragments with specific biological functions. Activation of complement cascade is finished by formation of a membrane-attacking complex associated with lysis of the target cell. The main functions of the complement system include opsonization, chemotaxis, and clearance of immunocomplexes and apoptotic cells.
Congenital disorders of the complement system are very rare diseases. The patients are prone to recurrent infections caused mainly by encapsulated microorganisms (pneumococcus, meningococcus, haemophilus and staphylococus) and most of them develop systemic autoimmune diseases (resembling systemic lupus erythematosus) during their lifetime. The secondary decrease of complement components may be caused by decreased liver production of complement components, excessive or prolonged activation of the immune system as a result of various immunopathological conditions or because of the formation of autoantibodies against the complement components. Basic therapeutic approaches include vaccination against meningococcus, pneumococcus and haemophilus and antibiotic prophylaxis.
Keywords:
complement system – inborn errors of complement system – encapsulated bacteria – autoimmune disorders
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Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2021 Issue 4
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