How do you test for complement deficiency?
- Laboratory Studies. One can screen for deficiencies in complement by performing the total serum classic hemolytic complement (CH50) test or the alternative hemolytic complement (AP50) test.
- Imaging Studies. No specific imaging studies are indicated.
- Other Tests.
When do you suspect a complement deficiency?
[5] Complement deficiency should also be suspected in a patient with recurrent sinopulmonary infection with normal humoral (antibody) immunity and with/without autoimmunity. Pyogenic infections and sepsis in children and neonates may be an indication of mannan-binding lectin deficiency.
What causes complement system deficiency?
The cause of complement deficiency is genetics (though cases of an acquired nature do exist post infection). The majority of complement deficiencies are inherited as autosomal recessive conditions, while properdin deficiency occurs through X-linked inheritance. MBL deficiency can be inherited by either manner.
What does complement test for?
A complement test is a blood test that measures the activity of a group of proteins in the bloodstream. These proteins make up the complement system, which is one part of the immune system. The complement system helps antibodies fight off infections and destroy substances that are foreign to the body.
What causes low C3 and C4 levels?
This is the result of an acquired disease. If your C3 and C4 levels are reduced, this may be a sign that you have lupus. With lupus, your total complement level may be slightly lower. Low C3 and C4 levels may also be a sign of alcoholic liver disease.
Is complement deficiency a primary immunodeficiency?
Complement deficiency is a form of primary immunodeficiency disorder. Deficiency in any component of the complement system can lead to immunocompromise and overwhelming infection and sepsis. Deficiency can be inherited or acquired and complete or partial. Acquired deficiency can be caused by infection.
What would happen if a person has a complement deficiency?
Deficiencies in the complement cascade can lead to overwhelming infection and sepsis. In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE).
What happens to complement levels in autoimmune disease?
Many autoimmune diseases are characterized by generation of autoantibodies that bind to host proteins or deposit within tissues as a component of immune complexes. The autoantibodies can activate the complement system, which can mediate tissue damage and trigger systemic inflammation.
What does it mean if Complement C3 is low?
Lower-than-normal levels of C3 proteins can be a sign of: An autoimmune disease such as psoriatic arthritis, Crohn’s disease, lupus or rheumatoid arthritis, or that you’re at a higher risk of developing an autoimmune disorder.