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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.<\/jats:p>","DOI":"10.1177\/0961203317709347","type":"journal-article","created":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T08:03:00Z","timestamp":1495094580000},"page":"1550-1555","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":18,"title":["Autoantibodies against complement components in systemic lupus erythematosus \u2013 role in the pathogenesis and clinical manifestations"],"prefix":"10.1177","volume":"26","author":[{"given":"M H","family":"Hristova","sequence":"first","affiliation":[{"name":"Clinic of Nephrology, University Hospital Alexandrovska, Medical University \u2013 Sofia, Bulgaria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V 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