Hypocomplementemia
| Complement deficiency | |
|---|---|
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| Complement pathway (normal) | |
| Specialty |
Hematology 
|
| Causes | Deficiency can be inherited or acquired |
| Diagnostic method | CH50 measurement, Plasma level |
| Treatment | Immunosuppressive therapy |
| Classification |
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|---|---|
| External resources |
Complement deficiency is an immunodeficiency of absent or suboptimal functioning of one of the complement system proteins. Because there are redundancies in the immune system, many complement disorders are never diagnosed, some studies estimated that less than 10% are identified.Hypocomplementemia may be used more generally to refer to decreased complement levels while secondary complement disorder means decreased complement levels that are not directly due to a genetic cause but secondary to another medical condition.
The following symptoms (signs) are consistent with complement deficiency in general:
Vaccinations for encapsulated organisms (e.g., Neisseria meningitidis and ) is crucial for preventing infections in complement deficiencies. Among the possible complications are the following:
The cause of complement deficiency is genetics (though cases of an acquired nature do exist post infection). The majority of complement deficiencies are autosomal recessive, while properdin deficiency could be X-linked inheritance, and finally MBL deficiency can be both.
Acquired hypocomplementemia may occur in the setting of bone infections (osteomyelitis), infection of the lining of the heart (endocarditis), and cryoglobulinemia. Systemic lupus erythematosus is associated with low C3 and C4Membranoproliferative glomerulonephritis usually has low C3.
The mechanism of complement deficiency consists of:
Among the diagnostic tests that can be done in determining if an individual has complement deficiencies is:
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