Title

Hereditary Angioedema, Acquired C1 Inhibitor Deficiency, and Angiotensin-Converting Enzyme Inhibitor–Associated Angioedema

Authoring Organizations

American College of Allergy, Asthma, and Immunology

American Academy of Allergy, Asthma & Immunology

Publication Month/Year

June 1, 2013

Last Updated Month/Year

October 17, 2024

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Document Objectives

In 2013, the United States Hereditary Angioedema Association Medical Advisory Board (US HAEA MAB) published recommendations for the management of hereditary angioedema (HAE) due to C1 inhibitor (C1INH) deficiency (HAE-C1INH). This document builds upon the 2013 paper, updating and expanding those recommendations to reflect the rapid progress during the intervening years and incorporating recommendations for HAE with normal C1INH (HAE-nl-C1INH).

Target Patient Population

Patients with hereditary angioedema (HAE)

Inclusion Criteria

Male, Female, Adolescent, Adult, Child, Infant, Older adult

Health Care Settings

Ambulatory, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Diagnosis, Treatment, Management, Prevention

Diseases/Conditions (MeSH)

D054179 - Angioedemas, Hereditary, D056828 - Hereditary Angioedema Type III, D056829 - Hereditary Angioedema Types I and II

Keywords

angioedema, hereditary angioedema, HAE, acquired C1 inhibitor deficiency, C1 Inhibitor Deficiency, C1INH deficiency

Source Citation

Busse PJ, Christiansen SC, Riedl MA, Banerji A, Bernstein JA, Castaldo AJ, Craig T, Davis-Lorton M, Frank MM, Li HH, Lumry WR, Zuraw BL. US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema. J Allergy Clin Immunol Pract. 2021 Jan;9(1):132-150.e3. doi: 10.1016/j.jaip.2020.08.046. Epub 2020 Sep 6. PMID: 32898710.