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Complement proteins are components of the innate immune system. There are 3 pathways to complement activation: 1) the classic pathway, 2) the alternative (or properdin) pathway, and 3) the lectin activation (mannan-binding protein; MBP) pathway. The classic pathway of the complement system is composed of a series of proteins that are activated in response to the presence of immune complexes. The activation process results in the generation of peptides that are chemotactic for neutrophils and that bind to immune complexes and complement receptors. The end result of the complement activation cascade is the formation of the lytic membrane attack complex (MAC).
Patients with deficiencies of the late complement proteins (C5, C6, C7, C8, and C9) are unable to form the MAC, and may have increased susceptibility to neisserial infections.
The majority of cases of C7 deficiency have neisserial infections, but cases of systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), scleroderma, and pyoderma gangrenosum have been reported. The pathogenesis of the rheumatic disease is not clear.
Complement levels can be detected by antigen assays that quantitate the amount of the protein. For most of the complement proteins, a small number of cases have been described in which the protein is present but is non-functional. These rare cases require a functional assay to detect the deficiency.
Diagnosis of C7 deficiency
Investigation of a patient with an undetectable total complement (CH50) level
Low levels of complement may be due to inherited deficiencies, acquired deficiencies, or due to complement consumption (eg, as a consequence of infectious or autoimmune processes).
Absent C7 levels in the presence of normal C3 and C4 values are consistent with a C7 deficiency. Absent C7 levels in the presence of low C3 and C4 values suggest complement consumption.
The total complement (CH50) assay (COM / Complement, Total, Serum) assay should be used as a screen for suspected complement deficiencies before ordering individual complement component assays. A deficiency of an individual component of the complement cascade will result in an undetectable total complement level.
Absent (or low) C7 functional levels in the presence of normal C7 antigen levels should be replicated with a new serum specimen to confirm that C7 inactivation did not occur during shipping.
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