oa Current Allergy & Clinical Immunology - Complement disorders in South Africa : diseases arising from genetically determined alterations or deficiencies of the protein components of the complement system : review article

Volume 20 Number 4
  • ISSN : 1609-3607



Diseases caused by deficiency or malfunction of the complement proteins, including the proteins that regulate the action of complement, are very diverse.

Some diseases are rare and have not been reported from South Africa. On the other hand there are a number of patients with deficiency of the control protein C1-esterase inhibitor and these patients suffer hereditary angioneurotic oedema (HANE). This disease requires management because the episodes of angio-oedema can be life-threatening.
A disease that is more common in the Western Cape than elsewhere is complete deficiency of the sixth component of complement (C6D). Like deficiency of the other late components (C5, C7, C8 and C9) it causes dramatically increased susceptibility to and patients present with repeated infections. The consequences of repeated episodes of meningococcal meningitis can be very serious and steps to prevent infections are required.
One disease that is a common cause of loss of central vision in the elderly is age-related macular degeneration (AMD). Recently it has been shown to be associated with a polymorphism of the complement regulator protein factor H. The rarer variant of factor H appears less efficient in regulating complement. We do not know of studies of this polymorphism in South African but it is likely that some of us carry the gene for the rare form and eventually some of us will be affected.

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