An overview of angioedema: Pathogenesis and causes
- Bruce Zuraw, MD
Bruce Zuraw, MD
- Professor of Medicine
- University of California San Diego
- Clifton O Bingham, III, MD
Clifton O Bingham, III, MD
- Associate Professor of Medicine
- Johns Hopkins University
Angioedema is self-limited, localized swelling of the skin or mucosal tissues, which results from extravasation of fluid into the interstitium due to a loss of vascular integrity. Angioedema may occur in isolation, accompanied by urticaria, or as a component of anaphylaxis.
The pathogenesis and causes of angioedema will be reviewed here. The clinical features, diagnosis, differential diagnosis, and management of acute angioedema are discussed separately. (See "An overview of angioedema: Clinical features, diagnosis, and management".)
Data regarding the epidemiology of angioedema are limited, although it affects both adults and children and is not a rare disorder. In a retrospective review of all hospital admissions in New York State over 13 years, angioedema was the second most common "allergic" disease to necessitate hospitalization, exceeded only by asthma . In this study, the number of angioedema hospitalizations per year more than doubled during the study period, suggesting that the prevalence may be increasing.
African Americans were disproportionately affected, as they accounted for 42 percent of the admissions for angioedema, but only 16 percent of the state's population.
The epidemiology of specific forms of angioedema, such as hereditary angioedema due to C1 inhibitor (C1-INH) deficiency, is discussed elsewhere. (See "Hereditary angioedema: Epidemiology, clinical manifestations, exacerbating factors, and prognosis".)
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- Mast cell-mediated etiologies
- - Allergic reactions
- - Direct mast cell release
- - Aspirin and NSAIDs
- - Chronic urticaria with or without angioedema
- Bradykinin-mediated etiologies
- - ACE inhibitors
- - Hereditary angioedema and acquired C1 inhibitor deficiency
- Etiologies of unknown mechanism
- - Idiopathic angioedema
- - Infections
- - Calcium channel blockers
- - Fibrinolytic agents
- - Other drugs
- - Herbal medicines
- - Other rare causes
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