Status of Dental Care Practices in Patients With Hereditary Angioedema

Hereditary angioedema (HAE) is a rare genetic disease, it can occur at any age and evolves in flare-ups. These are subcutaneous or submucosal edemas responsible for tightness and pain. All areas can be reached. In addition, all their life, patients have an unpredictable risk of death from asphyxiating angioedema (25% mortality in the absence of specific treatment).

Surgical procedures, endoscopies and dental procedures can trigger laryngeal AE. Thus, the national reference center for angioedema (CREAK) and the latest international consensus1 recommend that all these procedures be preceded by short-term prophylaxis. The reference treatment is the C1 inhibitor 2 concentrate. But to date, there is no prospective study that has demonstrated the effectiveness of this short-term prophylaxis before dental treatment. Only a retrospective study has shown that its implementation made it possible to reduce the risk of crisis by 42% after tooth extraction 3 and that the risk of crisis was greatest within 8 hours following tooth extraction.

The C1 inhibitor concentrate must be administered IVL by a nurse within 6 hours before the procedure and therefore implies, in addition to its high cost (1500 €), an organizational burden for the patient who must plan for the passage of an IDE, the availability of the product and this sometimes for a simple dental treatment.

These constraints have two consequences for dental care: patients avoid going to the dentist to the detriment of their dental health or do not take prophylaxis with the risk of a fatal attack.