Angioedema acquired T78.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Acquired quinckeedema; angioneurotic edema; Bannister's disease; Quincke edema acquired

Definition
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Differently defined disease group. Originally, the adjective "acquired" was used to refer to an "acquired C1-esterase inhibitor deficiency" and was thus used in contrast to hereditary angioedema (see below complement system). However, "acquired" angioedema in a broader sense also includes: Both forms of angioedema are listed as independent subgroups.

Classification
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  • Type I (acquired angioedema, AAE I): partly with lymphoproliferative malignant diseases, e.g. malignant lymphomas.
  • Type II (AAE II): like AAE I, detectable antibodies against C1-INH.

Etiopathogenesis
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  • Quantitative and/or functionalC1-esterase inhibitor deficiency. Pronounced C1q reduction.
  • Drug-induced, e.g. by ACE inhibitors, Ciclosporin A or other drugs (e.g. hawthorn extracts [Crataegutt] and others).
  • Further described are the formation of antibodies against the active site of theC1-inhibitor as well as the activation of the complement cascade by anti-idiotypic antibodies with excessive consumption (AAE type II).

Manifestation
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3.8% ofC1-esterase inhibitor deficiencies(angioedema) are acquired First manifestation in middle or old age (in contrast to hereditary angioedema), negative family history.

Clinical features
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Recurrent occurrence of cortisone- and antihistamine-insensitive angioedema over 48-72 hours. Abdominal symptoms and lethal course may occur. Associations with neoplasms such as lymphomas are also possible. Common occurrence of livedo racemosa and paraproteinemia without tumor detection and persistence during angioedema therapy are described in individual case reports. Regular check-ups to rule out lymphoma.

Laboratory
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DecreasedC1q (typical of acquired forms); in addition to reduced C1 esterase inhibitor deficiency (protein concentration and enzymatic activity),C2,C4 and CH-50 are also reduced. During therapy normalization or increase to subnormal values of complement fractions.C4 can be seen as an indicator for a successful therapy.

Therapy
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Literature
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  1. Kirkup ME et al (2003) Experience with tranexamic acid for nonheriditary angio-oedema and urticaria. Br J Dermatol 149 (Suppl 64): 25-26
  2. Nashan D et al (1995) Life-threatening angioedema due to acquired C1-inhibitor deficiency associated with paraproteinemia and livedo racemosa. Dermatologist 46: 339-342
  3. Nettis E (2003) Clinical and aetiological aspects in urticaria and angio-oedema. Br J Dermatol 148: 501-506
  4. Netzwerk aktuell (2010) Angioedema caused by hawthorn extract (Crataegutt Novo). Medication telegram 41: 54

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Last updated on: 29.10.2020