Properdin

Properdin is an important complement factor, essential for the functioning of the alternative activation pathway. Properdin deficiency, an X-linked recessive trait, has been regarded as rare. It is associated with a very high susceptibility to meningococcal disease. In certain categories of patients with meningococcal disease, the frequency of properdin deficiency is quite high and concurrent MBL deficiency may put these patients at particularly high risk. It would be advisable to screen these categories for properdin and MBL deficiency and offer meningococcal vaccination in cases of deficiency.