A patient with hereditary angioedema has recurrent episodes of non-pruritic, non-urticarial swelling of the face, extremities, and gastrointestinal tract. Which complement component is most likely deficient?
- A C1 inhibitor (C1-INH) ✓
- B C3
- C Factor H
- D Decay accelerating factor (CD55)
Explanation
Hereditary angioedema is caused by C1 inhibitor deficiency (autosomal dominant). Without C1-INH, uncontrolled kallikrein activation generates excess bradykinin, causing increased vascular permeability and angioedema. Unlike histamine-mediated angioedema, it is non-pruritic and does not respond to antihistamines. C3 deficiency causes recurrent infections. Factor H deficiency causes atypical HUS. CD55 deficiency causes paroxysmal nocturnal hemoglobinuria.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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