The international HAE guideline under real-life conditions: From possibilities to limits in daily life - current real-world data of 8 German angioedema centers

Background and objectives: Patients with rare diseases like hereditary angioedema (HAE) are usually referred to an angioedema center to ensure guideline-compliant and experience-based therapy. Even though there are established guidelines and several approved therapeutics, there are still open questions and situations in the daily care of HAE patients, where an exchange between centers is needed.

Materials and methods: A survey was conducted among physicians from German angioedema centers regarding challenges and issues in everyday HAE treatment. The main focus was on the topic of long-term prophylaxis (LTP). For rarer subcategories of angioedema, the centers conducted a literature review to discuss open questions.

Results: The responses of 12 physicians from 8 angioedema centers were analyzed in the survey. The attack frequency was the most important criterion for deciding to initiate LTP in HAE patients (100%). Two centers no longer generally recommend the initiation of pre-interventional prophylaxis in HAE patients under LTP. The therapeutic concepts of acquired angioedema due to C1 inhibitor deficiency and HAE in children were two associated specialized areas that were discussed in more detail.

Conclusion: The current guideline serves as the foundation for daily practice in treating HAE at specialized centers. Thus, for rare conditions like HAE, an exchange among the treating centers is essential to adequately address specific issues and rare subgroups.