Angioedema acquired/c1 esterase inhibitor deficiency T73.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Acquired angioedema at the base of an (acquired)C1-esterase inhibitor deficiency. Typical are decreased values forC1-esterase inhibitor, CH50, CC1q, CC1 and CC2; no heredity.

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Start of the disease in middle age.

Clinical features
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S.u. angioedema. An important difference to allergic or pseudoallergic angioedema is the absence of (accompanying) urticaria.

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In acute cases, slowly i.v. substitution ofC1-INH, repeat if necessary. Intensive medical treatment and monitoring depending on clinical appearance. Prophylactically, danazol and stanozole are used; in pregnancy and in children, tranexamic acid and epsilonaminocaproic acid (EACA) are particularly suitable. Dose and exact procedure see. Table 1.

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Treatment of angioedema at the base of a C1-esterase inhibitor deficiency

Acute therapy/prophylaxis

Therapy measures

Acute therapy

Analgesics for pain.

AddC1-INH(500-1000 units in 10 ml physiological NaCl solution), slowly i.v., the injection can be repeated depending on the clinical picture.

If available, 500-2000 ml fresh plasma or "fresh frozen plasma".

If indicated, intubation, tracheotomy or criotomy.

Caution: Adrenalin, glucocorticoids and antihistamines are ineffective!

Preoperative short-term prophylaxis

Danazol about 600 mg/day, 1-10 days before the procedure.

Alternatively: EACA approx. 6 g/day, 2-3 days before the procedure.

Alternatively:C1-INH approx. 500-1000 units i.v., shortly before the procedure.

Long term prophylaxis

Danazol at the beginning 600 mg/day, later reduction attempt to 200 mg/day or tranexamic acid 2-3 g/day (children: 1.5 g/day).

Alternatively: EACA 6 g/day (children: 2 g/day).

Alternatively:C1-INH 500 units i.v., every 4-5 days.

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Last updated on: 29.10.2020