The clinical suspicion is confirmed by measuring the C1-esterase inhibitor activity or the C1-esterase inhibitor antigen in the blood plasma.
There is currently no cure for patients suffering from HAE attacks. There is also no therapy to completely prevent the attacks. Nevertheless, the symptoms can be alleviated and the progression of the edema prevented. There are three types of therapy: long-term prophylaxis, short-term prophylaxis and the treatment of acute attacks.
Who needs long-term prophylaxis?
Long-term prophylaxis is not required for all patients with hereditary angioedema, but only for those in whom one or more of the following can be observed:
- the swelling of the mucous membranes affects the respiratory tract
- the attacks occur more than once a month
- the attack or illness is so serious that the patient can no longer participate in everyday life
First choice treatment option for hereditary angioedema
The administration of C1-esterase inhibitor concentrate has proven to be highly effective both in long-term prophylaxis and in acute therapy – particularly in patients with side effects on the previously used androgens. The enzyme that is missing to the patient is supplied directly by intravenous administration . Undesirable serious side effects are not yet known.
Other treatment options: androgens
Before C1-esterase inhibitor concentrate was the first choice, androgens were used. However, the multiple occurrence of benign liver tumors under the androgen danazol led to the substance being withdrawn from the market in Germany in 2005.
Androgens are male sex hormones and have two main properties: They increase protein production in the human organism and influence the development of male sex characteristics (e.g. increased hair growth, deeper voice).
Androgens and their effects on the organism
Androgens have been successfully used in the treatment of HAE in a dose between 50 to 200 mg / day, but can lead to virilization (“masculinization”) in women. In addition, they can cause irregularities in the menstrual cycle, weight gain and mood changes and have a negative impact on liver function.
Androgens such as Danazol can be obtained from international pharmacies, but it is advisable to initiate such treatment under supervision in an HAE treatment center. The use of androgens in children is contraindicated, as is in pregnancy and in women who want to have children.
Short-term prophylaxis and therapy of acute attacks of the HAE
Short-term prophylaxis is particularly important when patients with hereditary angioedema need surgery or dental treatment is required. In this case, the missing C1 esterase inhibitor enzyme can be administered in the form of an intravenous injection or infusion. The required dose is usually 500 to 1000 units of C1 esterase inhibitor concentrate.
In the acute case, the missing substance C1 esterase inhibitor concentrate is administered intravenously immediately. This causes the attack to end rapidly and the mucosal edema to recede. An acute HAE attack does not respond to standard therapy for histamine-mediated angioedema.