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Guidelines for management of hereditary angioedema: What is new? What is missing?

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Hereditary angioedema is an autosomal dominant disease that presents with recurrent episodic swelling of the submucosal and/or subcutaneous tissues of the cutaneous, gastrointestinal, and respiratory systems. Evaluation and treatment guidelines have been published nationally and internationally to aid the treating provider.


A review of the most cited and most recent updated guidelines was undertaken to review key points and to explore real-world feasibility of incorporating them into clinical practice. The International World Allergy Organization/European Academy of Allergy and Clinical Immunology (WAO/EAACI) Guideline for the Management of Angioedema - The 2017 Revision and Update, and the consensus reports from the Hereditary Angioedema International Working Group, the Joint Task Force on Practice Parameters focused practice parameter update, and the most recently updated US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema were reviewed and summarized.


Key points that have been consistent throughout the guidelines include recommendations for evaluation and classification of hereditary angioedema as well as evidence-based guidelines for treatment. Further attention is required on the evaluation and continuous assessment of the burden of illness and quality of life (QoL).


The guidelines for management of hereditary angioedema provide a framework for the clinician. However, the physician-patient dialog with regard to the patient disease experience, which includes attack frequency, severity, and Qol, must be continually assessed.
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Document Type: Research Article

Publication date: January 1, 2022

More about this publication?
  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

    The goal of the Proceedings is to publish articles with a predominantly clinical focus which directly impact quality of care for patients with allergic disease and asthma and by having the potential to directly impact the quality of patient care. AAP welcomes the submission of original works including peer-reviewed original research and clinical trial results. Additionally, as the official journal of the Eastern Allergy Conference (EAC), AAP will publish content from EAC poster sessions as well as review articles derived from EAC lectures.

    Featured topics include asthma, rhinitis, sinusitis, food allergies, allergic skin diseases, diagnostic techniques, allergens, and treatment modalities. Published material includes peer-reviewed original research, clinical trials and review articles.

    Articles marked "F" offer free full text for personal noncommercial use only.

    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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