How is complement deficiency diagnosed?

Primary Testing Initial evaluation for suspected complement deficiency is used to identify the affected pathway and should include testing for CP and TP activity (using the CH50 assay for total hemolytic complement) and AP activity (using the AH50 assay for alternative pathway hemolytic activity).

What is complement deficiency disorder?

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 clinical condition is associated with Properdin deficiency?

Properdin deficiency is a rare X-linked disease in which properdin, an important complement factor responsible for the stabilization of the alternative C3 convertase, is deficient. Affected individuals are susceptible to fulminant meningococcal disease.

What causes low complement levels?

Complement levels may be decreased due to increased consumption (because of increased activation) or, more rarely, a hereditary deficiency. Hereditary deficiency in one of the complement proteins will usually lead to a high frequency of recurrent microbial infections.

What does it mean if complement C3 is low?

If only your C3 complement level is low and all other complement components are normal, it’s usually because of an inherited component deficiency. This makes it more likely that you will develop certain autoimmune disorders. More often, you will have low levels of several complement components at once.

How does complement deficiency cause lupus?

The complement system involves both the innate and the adaptive immune systems and has important roles in the pathogenesis of SLE. Complement deficiencies within the classical pathway (C1q, C4 and C2) of activation predispose for development of the autoimmune disease SLE.

What causes low complement?

How is Correctdin deficiency treated?

Recent evidence suggests that properdin-deficient individuals respond well when immunized with the tetravalent polysaccharide meningococcal vaccine (A,C,Y,W135), generating anticapsular antibodies and bactericidal anti-meningococcal activity against serotypes covered by the vaccine which translates into a lower …

What is persistent complement component deficiency?

Description. Collapse Section. Complement component 2 deficiency is a disorder that causes the immune system to malfunction, resulting in a form of immunodeficiency. Immunodeficiencies are conditions in which the immune system is not able to protect the body effectively from foreign invaders such as bacteria and …

Why is complement low in lupus?

Low complement levels often signify active lupus, especially lupus nephritis. However, it is difficult to ascertain whether low complement levels are due to consumption during inflammation or due to an inherent deficiency of one or more alleles. Even more obfuscating, the two scenarios may exist in one individual.

When to test for complement deficiency in meningococcal disease?

Persistent complement component deficiencies (e.g., C3, C5-9, properdin, factor H, factor D) Clinicians should consider complement testing in patients with meningococcal disease. Meningococcal disease is often the first sign that a person has complement deficiency, which is a hereditary condition.

What are the clinical indications for complement deficiencies?

Clinical indications for possible complement deficiencies include recurrent mild or serious bacterial infections, autoimmune disease, or episodes of angioedema (a painless, but often dramatic, swelling under the skin, or swelling in the intestines, which can be extremely painful).

How are Complement deficiencies and properdin deficiencies inherited?

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.

Is there a cure for a complement deficiency?

Definitive treatment of complement deficiencies requires replacing the missing component of the cascade, either through direct infusion of the protein or through gene therapy. Because neither of these options is currently available, treatment of these patients focuses on managing the sequelae of the particular complement deficiencies.