Podcast – All Things Angioedema – LevelUp

The ACARE LevelUp Next Level 2026 program is supported by various pharmaceutical companies who have not influenced the content or the choice of speakers. A list of all program sponsors and partners can be found here.

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In this episode, Thomas welcomes Dr. Sabine Altrichter, urticaria expert and physician at Kepler University Hospital in Linz, Austria, to discuss a fascinating and underrecognized condition: cholinergic urticaria — especially in patients who also experience angioedema.

  • What makes cholinergic urticaria different from other urticaria types?
  • Why do some patients develop angioedema in addition to wheals?
  • How can we distinguish between cholinergic urticaria and exercise-induced anaphylaxis?
  • What are current and emerging treatment options?

Dr. Altrichter shares insights from clinical research and experience, including findings that show nearly half of cholinergic urticaria patients may develop angioedema. She explains the two main subtypes — allergy-related and sweat gland-related — and what this means for diagnosis and treatment. The discussion also covers new treatment options like anti-IgE therapies, mast cell-depleting agents, and drugs in the clinical pipeline like remibrutinib.

Join us for a practical and engaging conversation on how to better diagnose and manage this unique condition.

Key Learnings from the Episode

  • Cholinergic urticaria is triggered by body heating (e.g., exercise, sauna, passive warmth), leading to small, itchy wheals.
  • Nearly 50% of patients also experience angioedema, often around the eyes or lips, indicating more severe disease.
  • Patients with angioedema tend to have longer-lasting and more intense symptoms than those without.
  • Two subtypes are emerging: one IgE/allergy-related and another with sweat gland dysfunction.
  • Angioedema in cholinergic urticaria is not typically life-threatening, but may be accompanied by mild systemic symptoms.
  • Provocation testing (e.g., exercise or stair running) helps confirm diagnosis.
  • Antihistamines are first-line, but often insufficient in patients with angioedema.
  • Omalizumab (anti-IgE) is effective in about two-thirds of patients.
  • Mast cell-depleting therapies and remibrutinib are promising treatments in development.
  • Differentiating from exercise-induced anaphylaxis is key: the latter causes more unpredictable and systemic reactions.
  • Ongoing trials may reshape how we treat both mild and severe forms of the disease.

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In this episode, Thomas welcomes Felix Aulenbacher, a bioinformatician and statistician from ACARE Berlin, to discuss the role of data science, machine learning, and artificial intelligence (AI) in angioedema research.

They discuss:

  • How does data analysis help classify different types of angioedema?
  • What role do AI and machine learning play in identifying disease patterns?
  • How does the “10 Questions” tool assist in diagnosing angioedema?
  • How is AI revolutionizing medical research, and what are its limitations?

Felix shares insights on the intersection of bioinformatics and medicine, the challenges of medical data analysis, and how AI is transforming the field of angioedema research.

Key Learnings from the Episode

  • Bioinformatics plays a crucial role in medical research, analyzing large datasets to uncover hidden patterns in diseases.
  • Data standardization is essential—poorly formatted data can make analysis difficult and lead to incorrect conclusions.
  • Machine learning models like Random Forest help classify different types of angioedema based on patient questionnaires.
  • The “10 Questions” tool has been developed to quickly differentiate different types of angioedema including HAE, mast cell-mediated angioedema, and drug-induced angioedema.
  • AI can enhance data analysis, but it requires careful validation—incorrect use can lead to misinformation.
  • ChatGPT and AI tools assist with coding and data analysis, but human oversight is still essential.
  • AIDUOS, a ChatGPT-based tool, has been developed for urticaria research, relying on verified medical publications.
  • AI is not a threat to data analysts, but professionals must adapt to its evolving capabilities.

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In this episode, Thomas welcomes Dr. Fernanda Minafra, allergist and immunologist at the Federal University of Minas Gerais, Brazil, to discuss one of the most concerning aspects of hereditary angioedema (HAE): mortality.

  • How often do HAE-related deaths occur?
  • Why is undiagnosed HAE a major risk factor?
  • What do global studies say about mortality rates?
  • What can be done to reduce HAE deaths worldwide?

Dr. Minafra shares insights from her research, including a systematic review on HAE-related deaths and a study on mortality rates in Brazil. She highlights the urgent need for better diagnosis, access to treatment, and public health policies to prevent avoidable deaths.

Join us for this crucial discussion on why early diagnosis and proper treatment are key to saving lives.

Key Learnings from the Episode:

  • HAE-related deaths are often caused by asphyxiation due to laryngeal angioedema.
  • HAE mortality rates remain high in many parts of the world, especially in developing countries.
  • A systematic review found that 1 in 20 HAE patients has died from asphyxia.
  • Undiagnosed patients are at much higher risk of fatal outcomes.
  • Many death certificates fail to properly identify HAE as the cause of death.
  • HAE patients who die from asphyxiation typically lose 20 years of life expectancy.
  • Access to diagnostic tests and effective treatments is essential for preventing deaths.
  • Global collaboration through ACARE centers is needed to improve mortality data collection and patient outcomes.

Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here – Feedback form ATA

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In this episode, Thomas welcomes Dr. Jane Wong from Queen Mary Hospital, Hong Kong, to discuss a groundbreaking method for diagnosing hereditary angioedema (HAE) in regions with limited laboratory access. Traditional tests like complement C4, C1 inhibitor levels and C1 inhibitor function are crucial but not widely available in many parts of the world, delaying diagnosis and treatment.

They discuss:

🔹 What is dried blood spot (DBS) testing?

🔹 How does DBS compare to conventional lab tests?

🔹 Can DBS be used for large-scale screening and family testing?

🔹 What are the limitations and future applications of DBS?

Join us for an in-depth discussion on how this simple, cost-effective method can revolutionize HAE diagnosis and patient care worldwide.

Key Learnings from the Episode:

Traditional HAE testing (C4, C1 inhibitor levels and function) is limited in many regions, delaying diagnosis and treatment.

Dried blood spot (DBS) testing offers a reliable, cost-effective alternative, requiring only a few drops of blood.

DBS showed strong correlation with conventional lab results, making it a promising screening tool.

DBS allows for easy at-home or community-based sample collection, overcoming logistical barriers.

DBS can be used for family screening, identifying undiagnosed relatives in high-risk groups.

The method is stable and easy to transport, requiring minimal laboratory infrastructure.

More extensive studies are needed to validate DBS for broader population screening.

Future applications may include genetic testing for HAE with normal C1 inhibitor cases.

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In this episode, Dr. Thomas Buttgereit speaks with Dr. Adil Adatia about the latest findings on hereditary angioedema (HAE) in Canada, focusing on the challenges in diagnosing and treating patients with normal C1 inhibitor levels. They explore the differences between well-known genetic variants, discuss the average diagnostic delay of over 10 years, and examine treatment approaches, including long-term prophylaxis and the role of estrogen as a trigger. Additionally, they highlight the need for standardized guidelines in diagnosing and managing this condition to better treatment response.

Key Learnings from the Episode

The average diagnostic delay for hereditary angioedema with normal C1 inhibitor levels exceeds 10 years in Canada.

Patients with normal C1 inhibitor function often experience different triggers, with estrogen and stress being more prominent compared to other angioedema variants.

A significant heterogeneous group of patients exists, where genetic mutations have yet to be identified.

Many patients require multiple long-term prophylaxis treatments, indicating a more severe disease course.

Genetic testing has historically been limited, but recent efforts are increasing its role in diagnosis.

The lack of standardized diagnostic criteria across Canada makes it difficult to classify and treat patients uniformly.

Insurance coverage for treatments like omalizumab varies by province and county, making access to care inconsistent.

There is a need for clear definitions of treatment trials and treatment failures to improve clinical decision-making.

What comorbidities are seen in people with HAE? Dr Linda Sundler Björkman joins Dr Thomas Buttgereit to discuss her research on comorbidities in HAE, including methodology, key findings, and the need for further research.

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For more information about the Global Allergy and Asthma Excellence Network – ACARE network and its activities, please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Comorbidities in hereditary angioedema—A population-based cohort study; Increased risk of venous thromboembolism in young and middle-aged individuals with hereditary angioedema: A family study; Trends in treatments with disease-specific and interfering drugs in patients with hereditary angioedema in Sweden; and Rare connective tissue diseases in patients with C1-inhibitor deficiency hereditary angioedema: First evidence on prevalence and distribution from a large Italian cohort study.

How do we identify people with non-steroidal anti-inflammatory drug (NSAID)-induced angioedema? Dr Thomas Buttgereit welcomes Dr Stefan Cimbollek to discuss NSAID hypersensitivity in people with angioedema, including clinical manifestations and management strategies.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: An algorithm for the diagnosis and treatment of nonsteroidal anti-inflammatory drugs hypersensitivity, 2024 update; Approaches to the diagnosis and management of patients with a history of nonsteroidal anti-inflammatory drug-related urticaria and angioedema.

What is the emotional, physical, and economical impact of HAE on patient caregivers? Dr Ricardo Zwiener joins Dr Thomas Buttgereit to discuss the significant burden on the “invisible second patients” (caregivers) in HAE and share expert advice on how to extend support to caregivers.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

How effective are current management approaches for HAE in controlling the disease? HAE patient and physician Kathrin Schön offers her perspective as she joins Dr Thomas Buttgereit to discuss insights from a quality-of-life survey assessing the emotional, physical, and social impact of living with HAE.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

The Global Angioedema Forum 2024 presented an opportunity to bring together medical specialists, patients, and caregivers from around the world to share their experiences of the management and diagnosis of angioedema. Dr Thomas Buttgereit considers the most poignant discussion points from the event, while Dr Dharmagat Bhattarai summarises some of the challenges he faces working in a clinical setting with limited resources.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

What are the available treatment options for long-term prophylaxis in HAE, and why are more options being investigated? Dr Thomas Buttgereit discusses the latest treatment advances, including mechanism of action, efficacy and safety, with guest Professor Timothy Craig.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

How can emergency physicians quickly recognise HAE in patients who present with acute angioedema? Dr Thomas Buttgereit explores a new “easy-to-apply” triage tool with guest Dr Stephen Betschel, who, with his team in Toronto, developed this tool to enable rapid identification of HAE and appropriate intervention in the emergency room setting.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: Identification of the recently described plasminogen gene mutation p.Lys330Glu in a family from Northern Germany with hereditary angioedema; Screening for plasminogen mutations in hereditary angioedema patients; and Treatment of patients with hereditary angioedema with the c.988A>G (p.Lys330Glu) variant in the plasminogen gene.

What are the clinical signs, symptoms, and pathomechanisms of HAE caused by a plasminogen mutation, a recently identified cause of HAE with normal C1 inhibitor activity? Dr Thomas Buttgereit and Dr Andreas Recke discuss this rare type of recurrent HAE, which can manifest for the first time in older adults, and note how “this is atypical for a genetic disease – normally, genetic diseases are hard-wired”.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: Identification of the recently described plasminogen gene mutation p.Lys330Glu in a family from Northern Germany with hereditary angioedema; Screening for plasminogen mutations in hereditary angioedema patients; and Treatment of patients with hereditary angioedema with the c.988A>G (p.Lys330Glu) variant in the plasminogen gene.

Professor Marc Riedl, Dr Danny Cohn, and host Dr Thomas Buttgereit consider why “there are quite a few patients who delay their on-demand treatment”, and discuss orally active agents under investigation that may help to overcome the barriers to timely treatment.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: The international WAO/EAACI guideline for the management of hereditary angioedema; US HAEA medical advisory board 2020 guidelines for the management of hereditary angioedema; and Delayed on-demand treatment of hereditary angioedema attacks: Patient perceptions and associated barriers.

When is genetic testing required in the diagnostic workup of recurrent angioedema? Dr Thomas Buttgereit talks genetic tests for HAE with Professor Roger Colobran Oriol, covering the types of samples that can be used, current technologies, and how results are interpreted.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: Genetics in the management of HAE; C1 inhibitor mutations in HAE; and the expanding spectrum of mutations in HAE .

“The pain was unbearable.” Hear about Antonia’s experience of being diagnosed with HAE, the burden of symptoms and attacks, the unexpected silver linings, and why trust and safety are an important aspect of healthcare for people with HAE.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: World Allergy Organization Journal, British Society for Immunology, and European Medical Journal.

Dr Thomas Buttgereit welcomes Henrik Balle Boysen, President of HAI International (HAEi), to discuss the HAEi network, including its origins, initiatives and tools developed to support people with HAE.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: South African Virtual Angioedema Centre; University of Cape Town Lung Institute, Allergy and Immunology Unit; and Fresh frozen plasma for on-demand hereditary angioedema treatment in South Africa and Iran.

Associate Professor Jonny Peter joins Dr Thomas Buttgereit to discuss angioedema in South Africa, including the burden of disease for patients, the importance of patient education, and the success of advocacy for access to targeted treatments.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: South African Virtual Angioedema Centre; University of Cape Town Lung Institute, Allergy and Immunology Unit; and Fresh frozen plasma for on-demand hereditary angioedema treatment in South Africa and Iran.

Dr. Thomas Buttgereit asks Dr. Clemens Schöffl about EAACI Congress 2024 highlights, which include early data on outcomes of CRISPR-based gene editing of KLKB1 in people with HAE.

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For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: EAACI Congress 2024 – Breakthroughs in management of hereditary angioedema; CRISPR-Cas9 in vivo gene editing of KLKB1 for hereditary angioedema; Clinical experience with berotralstat in patients with hereditary angioedema with normal C1-esterase inhibitor: A commented case series.

Dr. Thomas Buttgereit welcomes Dr Teresa Caballero to discuss HAE in pregnant women, including the possible reasons for increased attacks and recommended treatment options.

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For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: How does pregnancy and type of delivery affect the clinical course of hereditary angioedema?; and Successful pregnancy outcome in a hereditary angioedema patient with previous pregnancy losses: A proposed delivery plan.

Professor Anete Grumach joins Dr Thomas Buttgereit to discuss factor XII mutation in people with HAE, including the reasons for the high prevalence in females and recommended genetic tests.

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For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Homozygosity for a factor XII mutation in one female and one male patient with hereditary angio-oedema; CpaA is a glycan-specific adamalysin-like protease secreted by Acinetobacter baumannii that inactivates coagulation factor XII; and Angioedema with normal complement studies: What do we know?

Dr Thomas Buttgereit welcomes Dr Philip Li to discuss the unmet needs of people with HAE in Asia, including the lack of consensus guidelines for the management of HAE, the lack of suitable diagnostic tools, and limited access to treatment.

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For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Concomitant medication in patients with bradykinin-mediated angioedema – there’s more than ACE inhibitors; The role of angiotensin receptor blockers in patients with angiotensin-converting enzyme inhibitor-induced angioedema; and Angiotensin II receptor blockers are safe in patients with prior angioedema related to angiotensin-converting enzyme inhibitors – a nationwide registry-based cohort study.

Ear, nose and throat specialist Dr Robin Lochbaum joins Dr Thomas Buttgereit to discuss angiotensin-converting enzyme (ACE) inhibitor‑induced angioedema, including information on the incidence and mechanism of action, and antihypertensive treatment options.

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For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Concomitant medication in patients with bradykinin-mediated angioedema – there’s more than ACE inhibitors; The role of angiotensin receptor blockers in patients with angiotensin-converting enzyme inhibitor-induced angioedema; and Angiotensin II receptor blockers are safe in patients with prior angioedema related to angiotensin-converting enzyme inhibitors – a nationwide registry-based cohort study.

“Should it be considered a part of chronic spontaneous urticaria?” Dr Thomas Buttgereit welcomes Dr Marta Ferrer Puga to talk about mast cell-mediated angioedema, including discussion of the international urticaria guidelines definition.

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Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.

For more information about the ACARE network and its activities please visit https://acare-network.com/.

“There are a lot of doctors that should know about angioedema.” Ear, nose and throat specialist Dr Janina Hahn joins Dr Thomas Buttgereit to discuss the importance of implementing an interdisciplinary approach in HAE, including tips on overcoming challenges.

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For more information about the ACARE network and its activities please visit https://acare-network.com/.

“Children should be informed about their illness according to their age.” Professor Henriette Farkas joins Dr Thomas Buttgereit to discuss angioedema in children, covering acute treatment options and the differing symptoms between adults and children.

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Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.

For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: P-49 Transmission patterns in C1-INH deficiency Hereditary Angioedema favours a wild-type male offspring: our experience at Chandigarh, India; Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent; Screening for type II hereditary angioedema; Transmission patterns of C1-INH deficiency hereditary angioedema favours a wild-type male offspring and Epidemiology, management and treatment access of hereditary angioedema in the Asia pacific region.

“I want to take an extra step, just to make sure that we never give up and provide them with the care that they deserve to receive.” Dr Danny Cohn joins Dr Thomas Buttgereit to talk about the role of an HAE specialist providing long-term, often multigenerational care, including the benefits offered by ACARE centres.

Listen on Apple Podcast or Spotify.

Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.

For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: P-49 Transmission patterns in C1-INH deficiency Hereditary Angioedema favours a wild-type male offspring: our experience at Chandigarh, India; Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent; Screening for type II hereditary angioedema; Transmission patterns of C1-INH deficiency hereditary angioedema favours a wild-type male offspring and Epidemiology, management and treatment access of hereditary angioedema in the Asia pacific region.

Dr Thomas Buttgereit is joined by Professor Ankur Kumar Jindal to discuss his experience as a paediatrician treating angioedema in India, covering the reoccurring challenges and how best to counsel families when discussing the risk of transmission to the next generation.

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Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.

. Additional resources relating to the topics discussed in this episode can be found here: P-49 Transmission patterns in C1-INH deficiency Hereditary Angioedema favours a wild-type male offspring: our experience at Chandigarh, India; Management of hereditary angioedema in resource-constrained settings: A consensus statement from Indian subcontinent; Screening for type II hereditary angioedema; Transmission patterns of C1-INH deficiency hereditary angioedema favours a wild-type male offspring and Epidemiology, management and treatment access of hereditary angioedema in the Asia pacific region.

Dr Thomas Buttgereit is joined by Julia Föll and Reinhardt Britz to discuss the responsibilities of working within an ACARE office, covering audit criteria and ongoing ACARE projects.

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Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.

For more information about the ACARE network and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Become an ACARE, ACARE LevelUp: The ACARE physician information and education platform and Programme for the identification of mutations in genes of patients with recurrent idiopathic or hereditary angioedema.

“In the past, they’ve used 72 different combinations of outcomes and outcome measures.” Remy Peterson joins Dr Thomas Buttgereit to discuss the need for the AURORA study, and shares the resulting consensus for acute treatment outcomes in hereditary angioedema (HAE).

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Do you have suggestions for future episodes? Please provide feedback and offer your suggestions for future topics and expert selection here.

For more information about the ACARE network and its activities please visit https://acare-network.com/.

Additional resources relating to the topics discussed in this episode can be found here: The registered study protocol: AcUte tReatment Outcomes in heReditary Angioedema (AURORA) and Outcome measures in randomized controlled studies of acute therapy for hereditary angioedema: A systematic review.

Join Dr Lauré Fijen and Dr Thomas Buttgereit as they discuss emerging angioedema treatments, covering efficacy, safety, frequency and route of administration. The experts also reflect on the possibility of a cure for hereditary angioedema (HAE).

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Join Professor Anna Valerieva and Dr Thomas Buttgereit as they discuss the burden of living with rare forms of angioedema, particularly hereditary angioedema (HAE). Drawing from her expertise as an allergologist at the Medical University of Sofia, Bulgaria, Professor Valerieva emphasises the importance of viewing patients holistically and considering their preferences. The experts also review how the introduction of new treatments has changed patients’ lives and minimised recurrent angioedema attacks.

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Join Dr Andrea Zanichelli and Dr Thomas Buttgereit as they discuss complex angioedema cases, including acquired angioedema diagnosed in later life. Drawing from his perspective as Head of the Angioedema Center in Milan, Italy, Dr Zanichelli explores how C1 and C4 inhibitor deficiencies may indicate a possible acquired angioedema diagnosis, common comorbidities due to this condition, and the current treatment options available.

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Join Dr. Antonia Scherer and Dr. Thomas Buttgereit as they discuss the impact of recurrent hereditary angioedema on the larynx. Drawing from her perspective as an ENT expert, Dr Scherer explores how laryngeal angioedema is assessed in the clinic, and the current treatment options available.

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Dr Thomas Buttgereit is joined by Dr Carolina Vera to discuss the use of attenuated androgens in the treatment of hereditary angioedema and their associated risks and side effects. Both exerts delve into the safety, ethical considerations and alternative treatment options to using androgens for the treatment of hereditary angioedema. Dr Vera concludes the discussion with an in-depth explanation of the SHAERPA project, focusing on the discontinuation of androgen use in individuals with angioedema, and the benefits this project can have in providing guidance on alternative medications.

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Professor Jason Fok joins Dr Thomas Buttgereit to discuss angioedema updates from the European Academy of Allergy and Clinical Immunology (EAACI) Congress 2023 and the role EAACI has in disseminating novel insights in angioedema treatment and diagnosis. Dr Buttgereit takes a deep dive into how data presented at EAACI congresses can be implemented into specialist ACARE clinics and the benefits the congress has in aiding clinicians to implement the latest guidelines for the benefit of patients.

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Dr Thomas Buttgereit is joined by Professor Markus Magerl, leader of the angioedema clinics at ACARE Berlin. Their conversation explores the clinical features of recurrent angioedema and the essential differential diagnoses that dermatologists must bear in mind when encountering facial swellings.

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Welcome to our first episode of the All Things Angioedema – Learning about Angioedema with Dr. Thomas Buttgereit Podcast
Dr Thomas Buttgereit welcomes the renowned urticaria specialist, Professor Marcus Maurer. Professor Maurer is the founding force behind both ACARE and UCARE, and spearheads the popular podcast, All Things Urticaria. The pair delve into the significance of angioedema across all areas of medicine and offer insights into the role and responsibilities of angioedemologists. They also shed light on the complexities of tackling angioedema, highlighting the invaluable role of the ACARE global network in tackling these challenges.

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