Abstrakt

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 treatment of HAE patients where an exchange among the centers is needed.
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 more in detail.
Conclusions: 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.

Autoreninformation

Autoren

• J. Greve¹
• R. Lochbaum¹
• S. Trainotti²
• E.-V. Ebert²
• T. Buttgereit³
• A. Scherer⁴
• L. Knipps^{4, 5}
• A. Smola⁶
• S. Volc⁷
• A. Recke⁸
• K.M. Duda⁹
• M. Sulk¹⁰
• J. Hahn¹

Abteilungen

• ¹ Abteilung für Oto-Rhino-Laryngologie, Kopf- und Halschirurgie, Universitätsklinikum Ulm,
• ² Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde, Technische Universität München, München,
• ³ Institut für Allergologie, Charité – Universitätsmedizin Berlin, korporatives Mitglied der Freien Universität Berlin und der Humboldt-Universität zu Berlin, Berlin,
• ⁴ Abteilung für Hals-Nasen-Ohrenheilkunde, Universitätsklinikum Düsseldorf, Düsseldorf,
• ⁵ Abteilung für Hals-Nasen-Ohrenheilkunde, Kopf- und Halschirurgie, St.-Josefs-Hospital Hagen, Universität Witten/Herdecke, Hagen,
• ⁶ Abteilung für Dermatologie, Universitätsklinikum Düsseldorf, Medizinische Fakultät, Heinrich-Heine-Universität Düsseldorf, Düsseldorf,
• ⁷ Abteilung für Dermatologie, Universität Tübingen, Tübingen,
• ⁸ Klinik für Dermatologie, Allergologie und Venerologie, Universität zu Lübeck, Lübeck,
• ⁹ Klinik für Dermatologie und Allergologie, Medizinische Hochschule Hannover, Hannover,
• ¹ 
• ⁰ Klinik für Dermatologie, Universität Münster, Münster

Adresse

Prof. Dr. Jens Greve
Abteilung für Oto-Rhino-Laryngologie, Kopf- und Halschirurgie
Universitätsklinikum Ulm
Frauensteige 12
89075 Ulm
Email: [email protected]

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