Acquired angioedema

Acquired angioedema

Acquired angioedema (AAE) is characterized by acquired deficiency of C1 inhibitor (C1-INH), hyperactivation of the classical pathway of human complement and angioedema symptoms mediated by bradykinin released by inappropriate activation of the contact-kinin system. Angioedema recurs at unpredictable intervals, lasts from two to five days and presents with edema of the skin (face, limbs, genital...

New perspectives in acquired angioedema.

tion is artificial, since many patients have lymphoproliferative diseases together with autoantibodies to C1-INH. Treatments for AAE are the same as those for HAE3, with the exception of anti-CD20 monoclonal antibodies, such as rituximab, which was recently introduced and is aimed at depleting B lymphocytes. A recent Danish article [3] summarized national data on nine patients with AAE, making ...

Acquired angioedema responding to rituximab.

Acquired angioedema: a new target for rituximab?

Acquired angioedema (AAE) is a rare disorder characterized by recurrent attacks, late onset of symptoms and a marked decrease of C1 levels. Pathogenetically it is caused by an accelerated consumption of C1-inhibitor (C1-INH), resulting in C1-INH deficiency and uncontrolled complement activation. We describe successful treatment of a patient with AEE due to C1-INH autoantibody, administering 4 w...

Update on laboratory tests for the diagnosis and differentiation of hereditary angioedema and acquired angioedema.

The importance of laboratory testing in the diagnosis of hereditary angioedema (HAE) has increased with the advent of new treatment options in recent years. It has been 50 years since HAE was linked to a decrease of C1INH (the inhibitor of complement enzyme, C1 esterase), a link that provided for the first laboratory test available for this disorder. HAE is subdivided into types that can be di...