Abstract
The complement system is an essential part of innate immunity, with vital interconnections with the adaptive immune system. The complement system has several interconnections with the adaptive immunity, e.g. via bridging antibody recognition to complement-dependent opsonization and lowering the threshold for antigenic B-cell stimulation and regulating T cells. The complement system is activated via the classical, lectin or alternative pathway, which are all initiated by different structures and by different mechanisms. Complement deficiencies, acquired or hereditary, have been recognized for almost all of the known components of the complement system. Acquired deficiency may be caused by infections or immune-complex disorders. Most inherited deficiencies of complement components are expressed in an autosomal recessive pattern. Primary complement deficiencies are in particular associated with an increased susceptibility to recurrent and invasive bacterial infections and with autoimmune disorders. Although complement deficiencies are uncommon in the general population, individuals with such deficiencies frequently suffer from serious and often life-threatening diseases, which need specific therapy, including vaccines and easy access to antibiotics. Therefore, patients with recurrent or invasive bacterial infections, certain kidney diseases, familial autoimmune disease or angioedema, should always be tested for complement deficiencies. If a complement deficiency is identified, management focuses on the associated disease, such as infection or autoimmunity. Prevention of infections by vaccination and immediate treatment with appropriate antibiotics are crucial. In some of these patients, antibiotic prophylaxis may be considered, and special attention should be given to vaccination against encapsulated organisms. Also, early recognition and management of autoimmune diseases are necessary.
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Mahmoudi, M., Nilsson, P.H., Mollnes, T.E., Roos, D., Sullivan, K.E. (2017). Complement Deficiencies. In: Rezaei, N., Aghamohammadi, A., Notarangelo, L. (eds) Primary Immunodeficiency Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-52909-6_8
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