How do we treat patients with hereditary angioedema
Section snippets
The approach
Hereditary angioedema (HAE) treatment is usually divided into three parts:
- 1.
long term prophylaxis,
- 2.
short term prophylaxis,
- 3.
treatment of acute attacks.
While parts 2 and 3 apply to any HAE patient, patients eligible for a long-term prophylaxis should be carefully selected. Criteria for this selection should take into account how much the disease affects the quality of life and/or carries risk of fatality on one side, and what are the risks related to long-term treatment on the other.
Long-term prophylaxis
The endpoint of this treatment is to render angioedema symptoms sporadic, i.e. approximately 2 attacks per year. Two classes of drugs have been proven to be effective in this type of prophylaxis: antifibrinolytic agents and androgen derivatives [3], [4], [5]. The first have been proposed for HAE due to their inhibitory activity on plasmin, a fibrinolytic enzyme that seems to be involved in the pathogenesis of attacks. Two preparations, epsilon-aminocaproic acid (EACA) and tranexamic acid (AMCA)
Short term prophylaxis
It is intended to guarantee absence of symptoms for a period of days or weeks. It is effectively performed using attenuated androgens that, given for a short time, do not raise safety problems even in children and in pregnant women during the last trimester. In our experience, HAE patients, after 1 week of danazol treatment on high doses (600 mg/day), are protected from swelling. Hence, we recommend that patients exposed to conditions that can trigger life threatening attacks (manipulation of
Treatment of acute attacks
We mentioned at the beginning the high mortality rate of HAE when appropriate treatment is not available. Deaths are due to laryngeal edema, which does not respond to corticosteroids and adrenaline. At present, this emergency can be overcome only by substitution therapy either with C1-Inh concentrate or with fresh frozen plasma [28], [29]. The first one has several advantages: (i) it can be kept at home by the patients and even self infused if needed; (ii) it has reduced risks of blood-borne
Drugs to be avoided in HAE patients
HAE is frequently misdiagnosed as an allergic condition and even when the correct diagnosis is made, physicians approaching these patients remain very concerned about using drugs. The axiom: “angioedema equals allergic drug reaction” is difficult to eradicate. As an example, many of our patients have been treated by the dentists without anesthesia, on the wrong, but firm belief, that angioedema results from allergy to anesthetics and not from the trauma. Hence, it is important to stress that
Conclusions
In this review we tried to share with the readers our clinical practice in treating HAE patients. We recognize, while giving therapeutic options to newly diagnosed patients that we are not just offering to them tools to fight a potentially lethal condition, but we are rather showing a way out from the feeling of uncertainty that marks their lives. A major complaint for HAE patients is the difficulty to find physicians who are adequately informed on the different aspects of their disease. In
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2013, Journal of Oral and Maxillofacial SurgeryCitation Excerpt :Several treatments have been published for acute attacks in patients with suspected or confirmed diagnosis of HAE. Treatment is divided into 3 categories: acute prophylaxis before triggers and procedures; acute treatment for attacks; long-term suppression.3,5,11 While short-term prophylaxis and treatment of acute attacks apply to any HAE patient, individuals who are eligible for long-term prophylaxis should be carefully selected.11