Fact-sheet: Angio edema
Updated on 04/25/2016 at 8:49 AM
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Definition
Clinical manifestations related to C1-esterase inhibitor deficiency.
Clinical features
Multiple involvement:
- laryngeal: dysphonia and risk of asphyxia
- digestive: typically transient, segmental, pseudo-surgical involvement of the small bowel or colon, manifesting as submucosal edema, often with an associated peritoneal reaction
- biliary: subicterus and cholestasis
- cerebral
- genitourinary
Triggering factors:
- physical trauma / surgery
- medication
- pregnancy
- strong emotion
Laboratory findings
Marked decrease in C1-esterase inhibitor.
CT
Digestive involvement:
- segmental
- small bowel > colon
- pseudo-surgical
- marked submucosal edema
- significant peritoneal reaction
- no scarring after an attack
Management
Diagnosis confirmed by C1-esterase assay.
Classification
Two forms exist:
- hereditary, the most common
- acquired, most often secondary to a lymphoproliferative disorder
