Miloš Jeseňák, Zuzana Rennerová, Peter Bánovčin
Complement system consists from more than 40 effective and regulatory plasmatic glycoproteins which can be divided into 9 main components (C1-9), their co-factors and regulatory proteins. Complement primary immunodeficiencies (PID) present approximately 2 % of all primary immunodeficiencies. The most frequent is deficiency of C2 component and mannose-binding lectin. The most important and evident clinical symptoms of complement PID are recurrent infections caused by encapsulated bacteria (especially meningococci and pneumococci) and autoimmune diseases (SLE-like syndrome). Other clinical symptoms and diseases associated with complement PID are atypical haemolytic-uremic syndrome, membranoproliferative glomeluronephritis, paroxysmal noctural haemoglobinuria and quite common hereditary angioedema. Heterogeneity of clinical symptoms underlines the complexity of complement system. Therapy (except of hereditary angioedema) is symptomatic – antibiotic treatment of infections (in some patient also prophylactic administration of antibiotics) and the treatment of autoimmune manifestations similarly to those patients without complement PID. The current therapy of hereditary angioedema consists of C1-inhibitor concentrate, recombinant C1-inhibitor, bradykinin receptor antagonist, inhibitor of kallikrein, androgens ot tranexame acid. The most important preventive approach to the patients with these immunodeficiencies is the vaccination against causal pathogens in the periods without acute infections – the vaccination against Haemophilus influenzae type b, Streptococcus pneumoniae and Neisseria meningitidis.