Treatment of hereditary angioedema
It is essential to bear in mind that hereditary angioedema is a potentially life-threatening disease, since swelling of the airways without adequate measures being taken can lead to rapid death by suffocation. Therefore, it is crucial to provide the patient with an emergency ID card, which should be carried with him/her at all times. In addition, patients and their relatives must be informed in detail about the possible symptoms and the measures to be taken in an acute case.
In most cases, initial treatment at a specialized treatment center is recommended. Treatment of swelling in acute cases is not always necessary. Slight swellings in the area of the hands and feet, for example, do not necessarily require treatment as long as they do not disturb the person affected.
The gastrointestinal attacks also do not necessarily need treatment. For moderate attacks, oral administration of an antispasmodic drug such as Buscopan ® may be sufficient. In some cases, however, the colicky symptoms are so painful that specific treatment is necessary.
In acute cases, a so-called C1-INH concentrate is administered. These are inhibitors of a certain factor (C1) of the immune system, which can reduce swelling. The concentrate has to be administered via a venous access, which can also be carried out independently through training.
Alternatively, the agent Icatiband is available. It is a so-called bradykinin antagonist that can be injected under the skin and inhibits the vasodilating hormone bradykinin. Patients with swelling of the mouth, throat or larynx are considered an emergency and must be admitted to hospital immediately.
In such cases, intubation may be necessary to secure the airways. If more than 12 attacks per year still occur despite adequate therapy, a prophylactic (preventive) measure should be considered. For this purpose, androgens such as danazol, oxandrolone and stanazolol may be considered, but they are not approved in Germany for the treatment of hereditary angioedema due to their numerous side effects.
Another drug for long-term prophylaxis is tranexamic acid, which is antifibrinolytic, i.e. it counteracts the dissolution of blood clots. A possible side effect is therefore the formation of blood clots (thromboses). Long-term treatment with C1-INH concentrate is also a possible therapeutic approach.
