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The complement system is an integral part of the immune defenses. It can be activated via immune complexes (classic pathway) or by bacterial polysaccharides (alternative pathway). The classic complement pathway consists of recognition, (C1q, C1r, C1s), activation (C2, C3, C4), and attack (C5, C6, C7, C8, C9) mechanisms with respect to their role in antibody-mediated cytolysis. C4 is 1 of the activation proteins of the classic pathway.
In the absence of C4, immune complexes will not be cleared by C3 activation peptides, but bacterial infections can still be defended via the alternative pathway.
C4 may be decreased in systemic lupus erythematosus, early glomerulonephritis, immune complex disease, cryoglobulinemia, hereditary angioedema, and congenital C4 deficiency.
Investigating an undetectable total complement (CH50)
Confirming hereditary angioedema (with low C1 inhibitor)
Assessing disease activity in systemic lupus erythematosus, proliferative glomerulonephritis, rheumatoid arthritis, and autoimmune hemolytic anemia
Decreased in acquired autoimmune disorders, in active phase of lupus erythematosus, and in rheumatoid arthritis
An undetectable C4 level (with normal C3) suggests a congenital C4 deficiency
Increased in patients with autoimmune hemolytic anemia
The results are dependent on appropriate specimen transport.
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