Father and son engaging in physical activity

Hereditary Angioedema

Hereditary angioedema (HAE) is an autosomal dominant genetic disease characterized by recurrent attacks of localized subcutaneous and mucosal swelling.1,2

Angioedema attacks most commonly affect the face, extremities, abdomen and urogenital area and are often painful and debilitating.1,2

Less commonly, they can occur in the laryngeal area causing airway obstruction and potentially becoming life-threatening.2

Inflammatory condition educational diagram

Epidemiology

HAE is a rare condition with an estimated prevalence of 1 in 50,000, though exact prevalence may vary due to misdiagnosis and geographical region.1,2,3 Symptoms are unpredictable and can manifest at any age, but typically appear during childhood (5 to 11 years), worsen during puberty (particularly among females), and persist throughout life.2,4,5,6,7 

Pathophysiology

HAE is an autosomal dominant disorder resulting from a deficiency of C1 inhibitor (C1-INH), which is a naturally occurring protein that inhibits plasma kallikrein, a key mediator of inflammation.1,8,9,10,11 C1-INH deficiency results in uncontrolled kallikrein activity, leading to increased generation of the vasoactive peptide bradykinin and associated symptoms of pain and swelling.8,9,10,11,12 

The disease is marked by its genetic variability – more than 510 different mutations of the C1-INH gene (SERPING1) have been identified.13 However, it is generally classified into 3 types according to the underlying cause and levels of C1-INH:
 

  • Type I (~85% of cases) is associated with low plasma levels of the C1-INH protein2,14,15
  • Type II (~15% of cases) is associated with a dysfunctional C1-INH protein despite normal plasma levels2,14,15
  • HAE with normal C1-inhibitor levels (formerly type III, estrogen-dependent) is very rare and is associated with normal C1-INH levels and function but has been linked to a number of other mutations2,14,15

Diagnosis

Due to its rarity and symptom overlap with other conditions, HAE is frequently under-recognized leading to delayed diagnosis.4,7,16,17 Historically, the rate of mortality has been higher in undiagnosed HAE patients due to lack of awareness of potential fatal laryngeal attacks and inadequate treatment.18 The two most common forms of HAE (types I/II) should be suspected when a patient presents with a history of recurrent angioedema attacks and further substantiated by:19 

  • Positive family history (may be absent in 25% of cases)
  • Onset of symptoms in childhood/adolescence
  • Recurrent and painful abdominal symptoms
  • Occurrence of upper airway edema
  • Failure to respond to antihistamines, glucocorticoids, or epinephrine
  • Presence of prodromal signs/symptoms before swellings
  • Absence of urticaria (wheals/hives)

To confirm diagnosis, patients should be assessed for blood levels of C1-INH protein, C4 and C1-INH function.19

Navigating HAE

The condition is associated with a substantial and multifaceted burden of illness affecting various aspects of a patient's quality of life (e.g., emotional state, career and education progression, and the decision to have children).2,7,20,21,22,23,24 Types I/II are indistinguishable in their clinical presentation, having identical symptoms characterized by edema attacks that can vary in location, frequency, duration, and severity.1,25,26 Swelling and other symptoms gradually worsen over 12 to 36 hours, intensifying in a relentless manner, sometimes spreading to other sites, and then resolving over 2 to 5 days.7,14,27

A variety of possible triggers for attacks have been proposed, most commonly mechanical trauma, mental stress, and airway infection.2,4,28 Nevertheless, many attacks occur without an obvious trigger, particularly in children, highlighting the need for individualized treatment plans.2,4,19

Medications

This resource provides information on Takeda medications available in the Hereditary Angioedema category and is not intended to represent a complete list of therapeutic options.

Cinryze®

[C1 Esterase Inhibitor (Human)]

Firazyr®

(icatibant)

Kalbitor®

(ecallantide)

Takhzyro®

(lanadelumab-flyo)

Scientific Congresses and Resources

This is not intended to be a comprehensive resource of all congresses and congress materials across therapeutic and disease areas. Congress materials may include information about investigational use(s) of compounds/products that are not approved for use by the U.S. Food and Drug Administration (FDA) and/or are inconsistent with the Prescribing Information. Takeda does not recommend the use of any Takeda product beyond the approved labeling. Any decisions regarding the usage of a Takeda product beyond the approved labeling are left to the discretion of the healthcare professional. Takeda makes no representations about whether investigational compounds or unapproved uses will be approved by the FDA.

American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM), 2025

October 29 - November 1, 2025 | Link to Event

Annual meeting dedicated to the advancement of neuromuscular, musculoskeletal, and electrodiagnostic medicine.

American College of Allergy, Asthma & Immunology (ACAAI), 2025

November 6 - 10, 2025 | Link to Event

Global meeting featuring thought leaders from around the world focused on advancing patient care in allergy and immunology.

Annual Meeting American Academy of Allergy Asthma and Immunology (AAAAI), 2026

February 27 - March 2, 2026 | Link to Event

Premier global educational event for allergists and immunologists, with thousands of attendees each year, discussing allergies, asthma, and immune deficiency disorders.

American Academy of Neurology (AAN), 2026

April 18 - 22, 2026 | Link to Event

Each year, the American Academy of Neurology's Annual Meeting offers a robust lineup of diverse learning opportunities covering nearly every topic and subspecialty, helping you stay up to date on the latest trusted science and essential education.

National Home Infusion Association (NHIA), 2026

April 18 - 22, 2026 | Link to Event

Brings together home and alternate site infusion professionals for four days of networking, education, and exhibits. The expo features companies displaying the latest products and services supporting the industry.

Immunoglobulin National Society (IgNS), 2025

October 16 - 19, 2025

National conference of IgNS bringing together professionals and practitioners from all disciplines and clinical specialties to advance Ig therapy practice, while providing networking and comprehensive education opportunities.

Hereditary Angioedema National Summit (HAEA), 2025

July 10 - 13, 2025

This event brings together over 1200 HAE community members, including families, caregivers, healthcare professionals, and pharmaceutical representatives.

Takhzyro® (lanadelumab-flyo)

  • Longitudinal Trend in Attack Outcomes in Patients With Hereditary Angioedema Receiving Long-term Prophylaxis with Lanadelumab: An Observational Study
  • Physician-Reported Attack Rates and Attack Characteristics Among Patients With Hereditary Angioedema Who Extended Dosing With Lanadelumab: An EMPOWER Study Analysis
  • Treatment Outcomes With Lanadelumab by Baseline Hereditary Angioedema Activity: EMPOWER/ENABLE Analysis

Eastern Allergy Conference (EAC), 2025

May 29 - June 1, 2025

Regional annual conference discussing the most current information from top experts in allergy, asthma, and immunology.

Takhzyro® (lanadelumab-flyo)

  • Lanadelumab’s Sustained Effectiveness and Safety for Hereditary Angioedema Long-Term Prophylaxis in Patients From Puerto Rico: Final Results From the EMPOWER Study
  • Understanding the Impact of Long-Term Prophylaxis Switches for Patients With Hereditary Angioedema.

American Thoracic Society (ATS), 2025

May 16 - 21, 2025

International conference showcasing the latest advances in pulmonary disease, critical illness, and sleep disorders for scientists and clinicians at all stages of their careers.

Glassia® [Alpha1- Proteinase Inhibitor (Human)]

  • Application of an Artificial Intelligence Model to Detect Alpha-1 Antitrypsin Deficiency: Characterizing the Study Population
  • Application of an Artificial Intelligence Model to Detect Alpha-1 Antitrypsin Deficiency: Model Performance

Clinical Immunology Society (CIS), 2025

May 1 - 4, 2025

Annual meeting with the goal of providing a stimulating forum with presentations and discussions on the latest advances in clinical immunology, including primary immunodeficiencies and immune dysregulatory diseases.

American Academy of Neurology (AAN), 2025

April 5 - 9, 2025

Annual meeting in neurology promoting high quality patient-centric care in a variety of topics and specialties through various learning formats.

  • Real-World Characteristics and Disease Management for a Virtual Longitudinal Cohort of Patients with Chronic Inflammatory Demyelinating Polyneuropathy

Academy of Managed Care Pharmacy (AMCP), 2025

March 31 - April 3, 2025

Large assembly of pharmacy and healthcare professionals dedicated to the issues of managed care pharmacy, offering immersive education sessions and keynote presentations.

  • Epidemiology, Patient Characteristics, Real-World Treatment Patterns, and Outcomes for Patient with Multifocal Motor Neuropathy (MMN)
  • Disease Severity and Healthcare Resource Utilization for Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy: Results from an Integrated Database

National Home Infusion Association (NHIA), 2025

March 29 - April 2, 2025

Annual conference ideal for infusion professionals or those seeking opportunities in the industry to come together and explore the latest trends in offering home-based services. 

Annual Meeting American Academy of Allergy Asthma and Immunology (AAAAI), 2025

February 28 - March 3, 2025

Premier global educational event for allergists and immunologists, with thousands of attendees each year, discussing allergies, asthma, and immune deficiency disorders.

Takhzyro® (lanadelumab-flyo)

  • Real-world Episodes of Long-term Prophylaxis for Hereditary Angioedema: A Descriptive Study Using Data From the Consortium of Independent Immunology Clinics
  • Longitudinal Study of Adult Patients With Hereditary Angioedema Receiving Long-term Prophylaxis for 3 Years or Longer With Lanadelumab: Baseline Findings
  • Understanding the Impact of Long-term Prophylaxis Switches for Patients With Hereditary Angioedema

Western Society of Allergy, Asthma and Immunology (WSAAI), 2025

February 9 - 13, 2025

Annual scientific session uniting allergists, immunologists, nurses, and physician assistants aimed at maintaining the highest standard of practice in allergy care.

Takhzyro® (lanadelumab-flyo)

  • Longitudinal Trend in Attack Outcomes in Patients With Hereditary Angioedema Receiving Long-term Prophylaxis With Lanadelumab: An Observational Study
  • Understanding the Impact of Long-term Prophylaxis Treatment Switches on Hereditary Angioedema Attack Characteristics

Cuvitru®[Immune Globulin Subcutaneous (Human)] 20% Solution

  • Clinical characteristics, treatment patterns, and healthcare resource utilization (HCRU) of patients with primary immunodeficiency diseases receiving immunoglobulin subcutaneous (human), 20% solution (Cuvitru) in a large US payer database

International Congress on Neuromuscular Diseases (ICNMD), 2024

October 25 - 29, 2024

This international congress offers attendees an updated view on neuromuscular disorders and networking opportunities to increase their international experience and collaborations.

American College of Allergy, Asthma & Immunology (ACAAI), 2024

October 24 - 28, 2024

Global meeting featuring thought leaders from around the world focused on advancing patient care in allergy and immunology.

Takhzyro® (lanadelumab-flyo)

  • Longitudinal Survey of Adult Patients with Hereditary Angioedema Receiving Long-Term Prophylaxis with Lanadelumab
  • Social Determinants of Health in Hereditary Angioedema and their Impact on Patient Outcomes

Videos

Watch videos focused on Hereditary Angioedema (HAE).

HAE Mechanism of Disease

Learn about Hereditary Angioedema (HAE), including the prevalence of the three types of HAE and their causes.

Consider Hereditary Angioedema in the Differential Diagnosis for Unexplained Recurring Abdominal Pain 

Learn about the delayed and misdiagnosis of Hereditary Angioedema (HAE) in patients presenting with abdominal symptoms.

 

Additional Resources

Find materials to help foster a deeper understanding of Hereditary Angioedema (HAE).

Hereditary Angioedema Disease Awareness Fact Sheet

An overview of HAE epidemiology, pathophysiology, signs and symptoms, diagnosis, and burden of disease.

Hereditary Angioedema Diagnosis Fact Sheet

An overview of HAE misdiagnosis, causes of angioedema, types of HAE, and diagnosing/testing for HAE.

Hereditary Angioedema nC1-INH Fact Sheet

An overview of HAE with nC1-INH including prevalence, pathophysiology, diagnosis, and subtypes.

Hereditary Angioedema in Pediatric Patients Fact Sheet

An overview of HAE in pediatric patients including diagnosis, clinical presentation, and disease burden.

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