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- Angioedema (AE) is acute, localized swelling of skin, mucosa, and submucosa caused by extravasation of fluid into the affected tissues.
- AE develops in minutes to hours and resolves in hours to days but can be life-threatening if the upper airway is involved.
- Two major classifications of AE exist:
- Histamine- or immune-mediated AE (HIAE) with urticarial
- Bradykinin-mediated AE without urticaria; both with unique subtypes
- Synonym(s): angioneurotic edema; Quincke edema
- Predominant age
- HIAE or idiopathic AE (IAE): any age
- Hereditary AE (HAE): age of onset infancy to 2nd decade of life
- Acquired AE (AAE): often patients >40 years
- Predominant gender: male = female (except HAE with normal compliment: women > men)
- AE occurs in 15% of the population over a lifetime.
- AE: 0.1–2.2% of patients receiving angiotensin-converting enzyme (ACE) inhibitors: African Americans have a 4 to 5 times greater risk of ACE inhibitor–induced AE than Caucasians.
- HAE: 1:10,000 to 50,000 population in the United States
Etiology and Pathophysiology
- HIAE with urticarial (1):
- Allergy to food (shellfish, nuts, eggs, milk, wheat soy), latex, venom, or medication (aspirin, NSAIDs, antibiotics, narcotics, and OCPs)
- Acute and chronic spontaneous urticaria
- Other urticarial/AE syndromes: cold urticaria, urticarial vasculitis, exercise-induced urticaria or anaphylaxis, episodic eosinophilia with AE, vibration-induced urticaria
- Bradykinin-mediated AE without urticarial (1):
- Attacks are triggered by prolonged mechanical pressure, cold, heat, trauma, emotional stress, menses, illness, and inflammation.
- HAE due to C1 INH deficiency
- Type I: the most common form, caused by decreased production of C1 esterase inhibitor (C1 INH)
- Type II: functionally impaired C1 INH
- HAE with normal C1 INH levels
- Formerly type III HAE
- Involves mutations in coagulation factor XII gene, often estrogen dependent, associated with estrogen administration
- AAE with C1 INH deficiency
- Rare condition
- Associated with lymphoproliferative disorders, autoimmune diseases (e.g., lupus)
- Type I: lack of anti–C1 INH autoantibody
- Type II: presence of anti–C1 INH autoantibody
- AE due to medications
- ACE inhibitors
- Dipeptidyl peptidase-4 inhibitors
- Direct renin inhibitor
- HAE types I and II are autosomal dominant, whereas HAE with normal C1 INH is dominant X-linked.
- HAE occurs in 25% of patients as a result of spontaneous genetic mutations.
- Consuming medications and foods that can cause allergic reactions
- Preexisting diagnosis of HAE or AAE
- Positive family history
- Avoid known triggers.
- Do not use ACE inhibitors in type I or II HAE.
Commonly Associated Conditions
- Quincke disease (AE of the uvula)