Renal disease associated with inherited disorders of the complement system

The human complement system is vital for host defense and plays a role in a number of inflammatory disorders. Inherited deficiency or dysfunction of most of the individual complement components occurs uncommonly. The phenotype displayed by such patients varies with the specific component deficiency and ranges from recurrent infections to autoimmune diseases. Most of the latter are associated with glomerulonephritis. The onset of severe lupus erythematosus in a young child, with prominent cutaneous and renal manifestations, especially if a similar disorder is present in another family member, is a clue to the presence of a complement component deficiency. The distinguishing of acquired deficiencies from inherited deficiencies in complement components is sometimes difficult and may require sophisticated laboratory testing.