Acquired Angioedema Type 1

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

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Drugs

C1-esterase inhibitor ( BERINERT, CINRYZE, HAEGARDA ), Ecallantide ( KALBITOR ), Icatibant ( FIRAZYR )

C1-esterase inhibitor ( BERINERT, CINRYZE, HAEGARDA ), Ecallantide ( KALBITOR ), Icatibant ( FIRAZYR ), Recombinant human C1-inhibitor, Recombinant inhibitor of human plasma kallikrein

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A type of acquired angioedema (AAE) characterized by acute edema in subcutaneous tissues, viscera and/or the upper airway.

Epidemiology

Prevalence is unknown.

Clinical description

Like other forms of AAE it has a later onset than HAE (see this term) and occurs generally in adults over the age of 50 where there is no family history of the disease.

Etiology

The disease occurs due to an acquired C1-INH deficiency. It is often associated with lymphoproliferative or autoimmune diseases which produce immune factors that destroy C1-INH leading to low levels of C1-INH, C1q complement and C4 complement.

Management and treatment

The treatments used for HAE can be effective for AAE but the most effective strategy is the treatment of the associated disease.