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This site is intended for U.S. Healthcare Professionals.
To report an adverse event or product quality complaint, call 1-877-TAKEDA-7 (1-877-825-3327)
Not actual patients.
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
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
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:
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
To confirm diagnosis, patients should be assessed for blood levels of C1-INH protein, C4 and C1-INH function.19
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
Medication Resources
[C1 Esterase Inhibitor (Human)]
(icatibant injection)
(ecallantide)
(lanadelumab-flyo)
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Biennial meeting that covers the latest discoveries in basic and clinical science covering our expanding field, bringing together internationally renowned scientists and clinicians to present and discuss major developments in diagnosis, clinical management, genetics, and immunobiology of inborn errors of immunity.
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The flagship meeting of the EAACI and the world’s largest congress specializing in the field of allergy and clinical immunology.
Regional annual conference discussing the most current information from top experts in allergy, asthma, and immunology.
International conference showcasing the latest advances in pulmonary disease, critical illness, and sleep disorders for scientists and clinicians at all stages of their careers.
Leading global conference discussing how to establish, incentivize, and share value sustainable for health systems, patients, and technology developers.
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Videos
Watch videos focused on Hereditary Angioedema (HAE).
Learn about Hereditary Angioedema (HAE), including the prevalence of the three types of HAE and their causes.
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).
An overview of HAE epidemiology, pathophysiology, signs and symptoms, diagnosis, and burden of disease.
An overview of HAE misdiagnosis, causes of angioedema, types of HAE, and diagnosing/testing for HAE.
An overview of HAE with nC1-INH including prevalence, pathophysiology, diagnosis, and subtypes.
An overview of HAE in pediatric patients including diagnosis, clinical presentation, and disease burden.