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Rare but Real: Tackling Acquired C1 -Inhibitor Deficiency
In this episode, Dr. Thomas Buttgereit welcomes Mats de Lange, medical doctor and PhD candidate at Amsterdam UMC, to discuss one of the rarest and most challenging forms of angioedema – acquired angioedema (AAE) due to C1 inhibitor deficiency (AAE-C1INH).
They discuss
🔹 What causes AAE, and how does it differ from hereditary angioedema?
🔹 Why is treatment still off-label for most patients?
🔹 What did the Amsterdam investigator-initiated trial reveal about Deucrictibant, a new bradykinin B2 receptor antagonist?
🔹 How could this research pave the way for the first approved AAE therapy?
Mats de Lange shares insights from his team’s investigator-initiated trial on the long-term use of Deucrictibant, showing remarkable results – three out of four patients remained attack-free for 20 months. The discussion also highlights the unique challenges of studying such an ultra-rare condition and the hope for future phase III trials.
Join us as we explore how collaborative research and patient participation are advancing the field of bradykinin-mediated angioedema.
Key Learnings from the Episode:
Acquired angioedema (AAE) is an extremely rare disorder caused by C1 inhibitor deficiency. Patients often have underlying hematologic diseases, making trials complex. Deucrictibant showed strong efficacy and safety in an investigator-initiated trial. All participants became attack-free during treatment, without major adverse events. Off-label access remains a challenge – approved therapies are urgently needed. Future multi-center studies and regulatory approval could transform patient care.
Chapters
00:00 Introduction to Angioedema and Guest Introduction
03:06 Understanding Acquired Angioedema due to C1 Inhibitor Deficiency
06:05 Current Treatment Landscape and Challenges
07:16 Investigator-Initiated Trials and Droycriptiband
11:05 Trial Design and Patient Characteristics
11:52 Promising Results from the Trial
13:06 Key Learnings and Future Directions
15:44 Next Steps in Research and Treatment Approval
Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.
Feedback form ATA: https://forms.office.com/e/ZWxx3D4Cmr
View all episodes
By ACARE, the Global Allergy and Asthma Excellence Network for AngioedemaIn this episode, Dr. Thomas Buttgereit welcomes Mats de Lange, medical doctor and PhD candidate at Amsterdam UMC, to discuss one of the rarest and most challenging forms of angioedema – acquired angioedema (AAE) due to C1 inhibitor deficiency (AAE-C1INH).
They discuss
🔹 What causes AAE, and how does it differ from hereditary angioedema?
🔹 Why is treatment still off-label for most patients?
🔹 What did the Amsterdam investigator-initiated trial reveal about Deucrictibant, a new bradykinin B2 receptor antagonist?
🔹 How could this research pave the way for the first approved AAE therapy?
Mats de Lange shares insights from his team’s investigator-initiated trial on the long-term use of Deucrictibant, showing remarkable results – three out of four patients remained attack-free for 20 months. The discussion also highlights the unique challenges of studying such an ultra-rare condition and the hope for future phase III trials.
Join us as we explore how collaborative research and patient participation are advancing the field of bradykinin-mediated angioedema.
Key Learnings from the Episode:
Acquired angioedema (AAE) is an extremely rare disorder caused by C1 inhibitor deficiency. Patients often have underlying hematologic diseases, making trials complex. Deucrictibant showed strong efficacy and safety in an investigator-initiated trial. All participants became attack-free during treatment, without major adverse events. Off-label access remains a challenge – approved therapies are urgently needed. Future multi-center studies and regulatory approval could transform patient care.
Chapters
00:00 Introduction to Angioedema and Guest Introduction
03:06 Understanding Acquired Angioedema due to C1 Inhibitor Deficiency
06:05 Current Treatment Landscape and Challenges
07:16 Investigator-Initiated Trials and Droycriptiband
11:05 Trial Design and Patient Characteristics
11:52 Promising Results from the Trial
13:06 Key Learnings and Future Directions
15:44 Next Steps in Research and Treatment Approval
Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.
Feedback form ATA: https://forms.office.com/e/ZWxx3D4Cmr