Angioedema

Question 1

What is Hereditary Angioedema (HAE)?

Answer 1

Autosomal dominant condition with low C1 (C1-INH) Protein.

Question 2

What is C1-INH?

Answer 2

Multifunctional serine protease inhibitor - uncontrolled release of bradykinin (oedema of tissues).

Question 3

Clinical Features of Angioedema (3).

Answer 3

1. Painful macular rash.
2. Painless, non-pruritic swelling of SC/sub-mucosal tissues.
3. Airways, Skin, Abdominal Organs.

Question 4

Investigations of Angioedema.

Answer 4

1. C1-INH : Low.

2. C2 and C4 : Low (C4 most reliable).

Question 5

Management of Angioedema (2).

Answer 5

1. Acute : IV C1-INH Concentrate or FFP (not Adrenaline, Antihistamine, Steroids).
2. Prophylaxis : Anabolic Steroid : Danazol.

Question 6

Pathophysiology of Angioedema (2).

Answer 6

1. Bradykinin = blood vessel dilation and increased permeability (angioedema).
2. C1 Esterase : Inhibits Bradykinin.