Caldwell et al. 1972; Alsenz et al. 1987.
Angioedema acquired/c1 esterase inhibitor deficiencyT73.3
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
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.
Occurrence/EpidemiologyThis section has been translated automatically.
The prevalence of acquired angioedema with C1-INH deficiency is unknown.
EtiopathogenesisThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
Onset of disease in middle or older age.
Clinical featuresThis section has been translated automatically.
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.
DiagnosticsThis section has been translated automatically.
C1-INH concentration
C1-INH activity (decreased)
C4(decreased)
C1q (decreased)
CH50
C1-INH antibody (see above)
TherapyThis section has been translated automatically.
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.
TablesThis section has been translated automatically.
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. |
LiteratureThis section has been translated automatically.
- 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