The clinic forms the South German Angioedema Centre with proven experts and in cooperation with the Technical University of Munich.
Angioedema (AE) is an oedematous swelling of the subcutaneous tissue. A rough distinction is made between histamine-mediated AE (e.g. in allergies, idiopathic urticaria), which is often accompanied by itching and wheal formation, and bradykinin-mediated angioedema, in which no itching occurs. In the latter forms, there is an increase in the tissue hormone bradykinin in the plasma. On the one hand, this can be caused by increased bradykinin production (hereditary angioedema = HAE) or by impaired degradation (drug-induced angioedema). HAE is usually caused by a lack of functional C1 esterase inhibitor, which inhibits bradykinin formation. Drug-induced AEs occur as a side effect of, for example, a group of blood pressure and heart medications, ACE inhibitors and sartans.
AEs preferentially occur in the lip, tongue, larynx, facial skin and extremities. Internal organs such as the intestines can also be affected. While AEs of the facial soft tissues are relatively unproblematic and gastrointestinal forms only rarely take a severe course, manifestations in the area of the base of the tongue and larynx can quickly escalate into a life-threatening situation due to the obstruction of the upper airways.
The treatment of bradykinin-induced AE differs fundamentally from that of allergic oedema or urticaria. The decisive factor is that corticosteroids or antihistamines have little or no effect.
Four drugs are authorised for the treatment of HAE in Germany. These are the C1 esterase inhibitor concentrates (C1-INH) Berinert®, Cinryze® and Ruconest®. Berinert® and Cinryze® are derived from human plasma. Ruconest® is a recombinant C1 esterase inhibitor. Berinert® and Ruconest® are authorised for acute therapy in Germany. Berinert® is also available as a preparation that is injected subcutaneously under the abdominal skin. In this form it is used preventively/prophylactically.
Another subcutaneously injected drug is the specific bradykinin B2 receptor antagonist Firazyr®. Firazyr® is administered subcutaneously from a pre-filled syringe and is authorised for the acute treatment of HAE.
Other drugs authorised for prophylactic therapy are Cinryze® and Takhzyro®. The latter is an antibody against plasma kallikrein, which leads to the release of the tissue hormone bradykinin in the blood plasma.
Although it is known that bradykinin plays a decisive role in the development of ACE inhibitor-induced AE, there is currently no therapy available that takes into account the mechanism of development of this oedema. Takhzyro® is administered subcutaneously every two to four weeks and has been shown in studies to relieve symptoms in the majority of patients with hereditary angioedema.
We offer a specialised consultation for patients who may be affected. Possible triggering factors are identified and appropriate therapy is initiated, which is monitored in the long term through regular check-ups. We also provide information on the latest treatment options. Please bring all medical documents such as medication or allergy passports with you.
Itching and wheals are more indicative of an allergic cause of the disease and are not usually symptoms of classic angioedema. While allergy-related skin changes and swelling can often be quickly alleviated by the use of cortisone and anti-allergic medication, angioedema as such does not respond to this therapy. In the case of itchy skin changes and suspected food intolerances or hives (so-called urticaria), an allergological-dermatological clinic/outpatient clinic should be consulted as a matter of priority in order to initiate further investigations.
Those affected suffer to varying degrees from sudden swelling in different parts of the body. Fluid leakage from the blood vessels into the surrounding tissue can lead to swelling in the abdominal area (associated with severe abdominal pain lasting up to several days), the trunk and extremities as well as in the upper respiratory and swallowing tracts - the latter can quickly lead to a life-threatening situation due to obstruction of the airways. The swelling can last for several days and occurs with varying frequency in those affected. They do not disappear after treatment with cortisone or anti-allergic medication. The swellings can occur out of the blue, but can also be induced by supposedly harmless medical procedures, such as dental treatment, or by injuries.
These swellings are never accompanied by itching or wheals!
As it is a hereditary disease, one parent or some of the siblings often have similar symptoms.
The disease is caused by a deficiency of a certain protein in the blood, the so-called C1 esterase inhibitor. Whether this substance is produced in sufficient quantities in the blood and is functioning normally can be tested in a special laboratory test. Only a few millilitres of blood need to be taken. You will then be informed of the results by post and, if there are any abnormalities, you will be advised about the treatment options in a detailed personal consultation. If the results are inconclusive, the blood sample must be taken again or a genetic analysis carried out.
Some blood pressure medications can also cause swelling that is not accompanied by itching or wheals and can last up to several days. These swellings occur almost exclusively in the head and neck area (tongue, throat, larynx) and do not respond to cortisone or anti-allergic medication.
Blood pressure medications that can trigger these swellings are so-called ACE inhibitors (their name often ends in "-pril") or so-called sartans.
The swelling often occurs after you have been taking the blood pressure medication for many months or even years.