C1-Esterase-Inhibitor Antibodies
C1 esterase inhibitor (C1-INH) antibodies are autoantibodies that target and inactivate C1-INH in the bloodstream. Autoantibodies are antibodies that mistakenly target and attack the body's own proteins or tissues, rather than foreign invaders such as viruses or bacteria.
In some individuals, the immune system produces C1-INH antibodies, which can lead to a rare autoimmune disorder called acquired angioedema (AAE). AAE is similar to hereditary angioedema (HAE), but in AAE, the deficiency of C1-INH is caused by the presence of these antibodies rather than a genetic mutation.
The presence of C1-INH antibodies in the blood reduces the levels and activity of functional C1-INH, leading to uncontrolled activation of the complement system and the generation of inflammatory mediators. This can cause recurrent episodes of swelling in various parts of the body, similar to HAE.
AAE can be treated with replacement therapy with C1-INH or other medications that target the complement system or inflammatory mediators, similar to HAE. However, the presence of C1-INH antibodies can make it more challenging to achieve effective control of symptoms, and treatment may need to be individualized based on the specific characteristics of each patient's disease.
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