All Things Angioedema

Episode 29 – Global Angioedema Forum 2024

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here:

Episode 28 – Emerging treatment options for long-term prophylaxis in HAE

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: C1 inhibitor review; open label extension of APeX-2 study; VANGUARD phase 3 study; HELP OLE study; OASIS-HAE study; CRISPR in HAE.

Episode 27 – Emergency room care: A rapid HAE triage tool

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Development of the hereditary angioedema rapid triage tool; Could it be hereditary angioedema?—Perspectives from different medical specialties.

Episode 26 – HAE and plasminogen mutation

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”.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 25 – Acute treatment of HAE attacks

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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 24 – Genetic testing in HAE: Behind the scenes

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.

For more information about the GA²LEN Global Network of Excellence 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 .

Episode 23 – A patient’s perspective on living with 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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 22 – HAE International

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: HAE International; HAE day; and ACARE.

Episode 21 – Angioedema: A South African perspective

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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 20 – EAACI 2024 reflections

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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 18 – Factor XII mutation in HAE

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.

For more information about the GA²LEN Global Network of Excellence 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?

Episode 17 – Unmet needs for HAE in Asia

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Epidemiology, management and treatment access of hereditary angioedema in the Asia pacific region; Screening for type II hereditary angioedema; and Hong Kong-Macau severe hives and angioedema referral pathway.

Episode 16 – ACE inhibitor-induced angioedema

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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 15 – Mast cell-mediated angioedema

“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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: A comparative study of sex distribution, autoimmunity, blood, and inflammatory parameters in chronic spontaneous urticaria with angioedema and chronic histaminergic angioedema; Angioedema severity and impact on quality of life: Chronic histaminergic angioedema versus chronic spontaneous urticaria; and Medical algorithm: Diagnosis and management of histaminergic angioedema.

Episode 14 – Collaboration in HAE

“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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/.

Episode 13 – Angioedema in the paediatric population

“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.  

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Results from the phase 3 SPRING study; International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency; and Diagnosing pediatric patients with hereditary C1-inhibitor deficiency.

Episode 12 – The HAE specialist: ‘Never give up’

“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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: The Chronic Urticaria Registry (CURE).

Episode 11 – The perspective from India in treating paediatric patients 

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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 10 – Who is behind the scenes of ACARE?

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.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 9 – The AURORA study

“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 HAE.

For more information about the GA²LEN Global Network of Excellence 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.

Episode 8 – The future of angioedema: Is there a cure on the horizon? 

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 HAE.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Hereditary angioedema: The clinical picture of excessive contact activation and Current and prospective targets of pharmacologic treatment of hereditary angioedema types 1 and 2.

Episode 7 – Treating hereditary angioedema

Join Professor Anna Valerieva and Dr Thomas Buttgereit as they discuss the burden of living with rare forms of angioedema, particularly 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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Mitigating disparity in healthcare resources between countries for management of hereditary angioedema and Therapeutic management of hereditary angioedema: past, present, and future.

Episode 6 – Tackling acquired angioedema and related comorbidities

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Diagnosis, course, and management of angioedema in patients with acquired C1-inhibitor deficiency, A nationwide study of acquired C1-inhibitor deficiency in France: characteristics and treatment responses in 92 patients, Icatibant treatment for acquired C1-inhibitor deficiency: a real-world observational study, Acquired C1-inhibitor deficiency and lymphoproliferative disorders: a tight relationship and Splenic marginal zone lymphomas in acquired C1-inhibitor deficiency: clinical and molecular characterization.

Episode 5 – An ENT view of angioedema

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.

For more information about the GA²LEN 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—The 2021 revision and update and Efficacy of Different Medical Therapies for the Treatment of Acute Laryngeal Attacks of Hereditary Angioedema due to C1-esterase Inhibitor Deficiency

Episode 4 – Androgens in hereditary angioedema

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.

For more information about the GA²LEN Global Network of Excellence 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—The 2021 revision and update, Attenuated androgen discontinuation in patients with hereditary angioedema: a commented case series and WAO Guideline for the Management of Hereditary Angioedema.

Episode 3 – Angioedema and EAACI 2023

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Biomarkers in hereditary angioedema, Managing diagnosis, treatment, and burden of disease in hereditary angioedema patients with normal C1-esterase inhibitor, The expanding spectrum of mutations in hereditary angioedema, and Recurrent angioedema: Diagnosing the rare and the frequent.

Episode 2 – What is angioedema and what is not?

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group.

Episode 1 – What “angioedemologists” do and what motivates them

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.

For more information about the GA²LEN Global Network of Excellence and its activities please visit https://acare-network.com/. Additional resources relating to the topics discussed in this episode can be found here: Definition, aims, and implementation of GA2LEN/HAEi Angioedema Centers of Reference and Excellence.