Hereditary Angioedema (HAE) is an autosomal dominant disease characterized by recurrent swelling throughout the body, including in the extremities, the upper airway, and the intestinal tract. The swelling has no known main trigger, although recognized triggers include infections, stress, surgical procedures, and trauma. HAE affects an estimated 1 in 50,000 individuals, with symptoms typically presenting during childhood or adolescence. 

The swelling attacks usually last a few days, and while an upper airway attack is the most concerning form, such instances account for a small percentage of overall attacks.

Today, we have the latest clinical guidelines on HAE, along with the most recent FDA approvals and ongoing clinical trials related to HAE that are anticipated to potentially impact future guideline updates.

Current Hereditary Angioedema Guidelines

Management of Hereditary Angioedema

  • European Academy of Allergy and Clinical Immunology (EAACI)
  • Published: January 2022

Management of Hereditary Angioedema

  • American College of Allergy, Asthma, and Immunology (ACAAI) / American Academy of Allergy, Asthma & Immunology (AAAAI)
  • Published: January 2021
Recent FDA Approvals for the Prevention and Treatment of Hereditary Angioedema

Below are the most recently approved FDA prevention and treatment options for HAE that we anticipate being incorporated into future clinical guidelines. 

Dawnzera (donidalorsen)

  • Ionis
  • Approved: August 2025
  • In August 2025, Dawnzera (donidalorsen), a prekallikrein-directed antisense oligonucleotide, was approved for adults and pediatric patients 12 years of age and older as prophylaxis to prevent HAE attacks. Dawnzera is the first RNA-targeted medicine approved for HAE.

Ekterly (sebetralstat)

  • KalVista
  • Approved: July 2025
  • Ekterly (sebetralstat) was approved in July 2025 for adults and pediatric patients 12 and older. Ekterly is a plasma kallikrein inhibitor that is the first on-demand treatment for HAE attacks, allowing patients to treat attacks as soon as they notice symptoms.

Andembry (garadacimab-gxii)

  • CSL Behring
  • Approved: June 2025
  • Andembry (garadacimab-gxii), an activated Factor XII inhibitor, was approved in June 2025 for adults and pediatric patients 12 and older as prophylaxis to prevent HAE attacks.

Takhzyro (lanadelumab-flyo)

  • Takeda
  • Approved: February 2023
  • In February 2025, Takzyro (lanadelumab-flyo), a plasma kallikrein inhibitor, was approved for pediatric patients 2 years of age and older to prevent HAE attacks. Previously, Takhzyro was approved for adults in 2018.
Ongoing Clinical Trials Related to Hereditary Angioedema

The following is a selection of ongoing, U.S.-based Phase 3 clinical trials based in the United States that we anticipate may impact future HAE clinical guidelines.

CSL312_3003 Safety and Pharmacokinetic Study in Subjects 2 to 11 Years of Age with Hereditary Angioedema

  • CSL Behring
  • Anticipated Primary Completion: December 2025

Study of Oral Deucrictibant Soft Capsule for On-Demand Treatment of Angioedema Attacks in Adolescents and Adults with Hereditary Angioedema

  • Pharvaris
  • Anticipated Primary Completion: March 2026

Open-Label Safety, PK, and Efficacy Trail of Sebetralstat (KVD900) in Pediatric Patients (Ages 2-11) With HAE Type I or II

  • KalVista
  • Anticipated Primary Completion: August 2027

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