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Caused by deficiency in C1 esterase inhibitor
80%
247/309
Caused by deficiency in C3
6%
18/309
Caused by deficiency in C5-C8
17/309
Caused by deficiency in decay accelerating factor
5%
15/309
Danazol is contraindicated in this disease as it may worsen symptoms
2%
6/309
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Hereditary angioedema is due to a deficiency in C1 esterase inhibitor, which results in elevated levels of edema-producing factors C2b and bradykinin. Hereditary angioedema is marked by a deficiency in C1 esterase inhibitor. The disease may affect the eyelids, lips, tongue, genitals, hands, and feet. There is localized edema, puffy skin, and swelling. In severe cases, there will be airway obstruction. ACE inhibitors are contraindicated in this disease. However, danazol, a synthetic androgen and partial agonist at the androgen receptor, plays a role in treatment. The condition is autosomal dominant. Muller discusses urticaria and angioedema. While urticaria is characterized by pruritic, raised wheals, angioedema is characterized by deep mucocutaneous swelling. In contrast to angioedema, urticaria is often self limited. The mainstay of therapy for urticaria is avoidance of triggering agents, oral corticosteroids, antihistamines, H2-receptor antagonists, tricyclic antidepressants, and leukotriene antagonists. Bork et al. discuss treatment with C1-esterase inhibitor (C1-INH) concentrate in hereditary angioedema. In this meta-analysis, it was found that replacement therapy with C1-INH significantly shortened time to onset of symptom relief in hereditary angioedema attacks compared with placebo in randomized controlled trials and was accompanied by an improvement in quality of life. The treatment was also well tolerated and safe. Image A depicts the classic facial appearance of a patient with hereditary angioedema. Illustration A depicts the complement pathway. Incorrect Answers: Answer 2: C3 deficiency may result in recurrent pyogenic sinus and respiratory tract infections. It also increases susceptibility to type III hypersensitivity reactions. Answer 3: C5-C8 deficiency results in recurrent Neisseria bacteremia. Answer 4: A lack of decay accelerating factor (DAF) results in complement mediated lysis of red blood cells and paroxysmal nocturnal hemoglobinuria. Answer 5: Danazol plays a role in treatment and is not contraindicated.
4.7
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