Acquired C1 Inhibitor Deficiency (C1-INH-AAE)

Acquired angioedema due to C1-INH deficiency (C1-INH-AAE) can occur when there are acquired (not inherited) deficiencies of C1-INH.

A quantitative or functional C1-INH deficiency with negative family history and low C1q is diagnostic of C1-INH-AAE.

The most common conditions associated with C1-INH-AAE are:

- autoimmunity
- B-cell lymphoproliferative disorders

A diagnosis of C1-INH-AAE can precede a diagnosis of lymphoproliferative disease and confers an increased risk for developing non-Hodgkin lymphoma.

Treatment focuses on symptom control with:

- therapies that regulate bradykinin activity (C1-INH concentrate, icatibant, ecallantide, tranexamic acid, androgens)
- treatment of any underlying conditions


Acquired C1 Inhibitor Deficiency

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