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