Abstract

Background: Hereditary angioedema (HAE) is a rare, potentially life-threatening disease caused in most cases by C1 inhibitor deficiency. Lanadelumab, a monoclonal antibody targeting plasma kallikrein, is an effective long-term prophylactic (LTP) treatment for HAE. However, consensus on best practices remains lacking. Objectives: This study aimed to report consensus statements on key principles on long-term lanadelumab therapy for HAE in Germany developed at an HAE LTP Expert Meeting in the year 2024 in Frankfurt, Germany. Materials and methods: A multidisciplinary panel of seven German HAE experts participated in a consensus process to align current guidelines with real-world clinical practice. Following literature review and debate, keynotes were drafted, refined, and voted on. Consensus was defined as ≥ 70% agreement. Key domains included: shared decision-making; flexibility in initiating or adjusting prophylaxis; structured patient education; self-administration; individualized dosing; emergency medication availability; and proactive follow-up, including specific guidance for women of childbearing age. Results: Ten core consensus statements were developed, achieving unanimous (100%, n = 9/10 statements) or strong (≥ 85%, n = 1/10 statements) agreement. It is recommended that the decision to initiate long-term prophylaxis be made through shared decision-making and that the decision made can and should be adjusted again in the further course of treatment. It is advisable to train patients in the technique of self-injection and to start therapy with lanadelumab with a 2-week injection interval in accordance with the product information. The injection interval should be adjusted to the individual patient, and all well-controlled patients should be offered the option of extending the interval without compromising the goal of complete disease control. Even and especially when the prophylaxis is well tolerated, emergency medication must not be neglected. Conclusion: These consensus statements provide a practical, expert-endorsed framework for implementing lanadelumab LTP in clinical practice emphasizing individualized treatment aligned with international guidelines and patient needs.

Author Details

Authors

• Emel Aygören-Pürsün¹
• Jens Greve²
• Inmaculada Martinez-Saguer³
• Susanne Trainotti⁴
• Mathias Sulk⁵
• Bettina Wedi⁶
• Ellen Witte-Händel⁷,⁸
• Markus Magerl⁷,⁹

Departments

• ¹ University Hospital Frankfurt, Goethe University, Frankfurt,
• ² Department of Otorhinolaryngology, Head and Neck Surgery, Ulm University Medical Center, Ulm,
• ³ Haemophilia Centre Rhine Main, Frankfurt/Main,
• ⁴ Technical University of Munich, TUM School of Medicine and Health, Department of Otorhinolaryngology, Head and Neck Surgery, TUM University Hospital, Munich,
• ⁵ Department of Dermatology, University of Münster, Münster,
• ⁶ Department of Dermatology and Allergy, Comprehensive Allergy Center, Hannover Medical School, Hannover,
• ⁷ Angioedema Center of Reference and Excellence (ACARE), Institute of Allergology, Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin,
• ⁸ Global Allergy and Asthma Excellence Network, ACARE/UCARE coordinating office, and
• ⁹ Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, Immunology and Allergology, Berlin, Germany

Address

Markus Magerl, MD, Charité – Universitätsmedizin Berlin, Institute of Allergology, Hindenburgdamm 27, 12203 Berlin, Germany
Email: [email protected]

Citation