Angioedema acquired/c1 esterase inhibitor deficiency T73.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 24.08.2022

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Caldwell et al. 1972; Alsenz et al. 1987.

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Acquired angioedema due toC1-esterase inhibitor deficiency. Typically, in type 1 of this angioedema group, there are decreased levels ofC1-esterase inhibitor, due to increased catabolism of the esterase inhibitor. There is no heredity (differentiation from hereditary angioedema (HAE). In type 2 (autoimmune type), there is formation of antibodies against C1-INH.

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The prevalence of acquired angioedema with C1-INH deficiency is unknown.

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Type1) In this type of angioedema, there is increased catabolism of -C1-INH. Frequently, lymphoproliferative diseases affecting B lymphocytes (non-Hodgkin's lymphoma or monoclonal gammopathies, including lupus erythematosus).The production of immune complexes results in complement activation with consumption of C1q and increased fixation of C1-INH.

Type2) In the 2nd type (autoimmune type) there is formation of autoantibodies against C1-INH without underlying disease.

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Onset of disease in middle or older age.

Clinical features
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See below Angioedema. The clinical picture of acquired bradykinin-mediated angioedema with C1 esterase deficiency is similar to that of HAE with C1 esterase deficiency. An important difference from allergic or pseudoallergic angioedema is the absence of (concomitant) urticaria.

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C1-INH concentration

C1-INH activity (decreased)


C1q (decreased)


C1-INH antibody (see above)

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In acute cases, antihistamines, glucocorticoids, or choroidal epinephrine are ineffective.

In the case of type 1, the triggering underlying disease must be treated.

In autoimmune type 2, immunosuppressive therapy may be successful.

Otherwise, treatment is the same as for hereditary C1-INH deficiency.

  • Substitution ofC1-INH slowly i.v., repeat if necessary. Intensive medical treatment and monitoring depending on the clinical presentation. Danazol and stanozol can be used prophylactically, and tranexamic acid and epsilonaminocaproic acid (EACA) in pregnancy and in children. 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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  1. Wedi B (2018) Urticaria and angioedema. In: G.Plewig et al (eds) Braun-Falco`s dermatology, venereology and allergology. Springer Reference Medicine SS 479-501

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Last updated on: 24.08.2022