AAAAI/ACAAI Hereditary Angioedema, Acquired C1 Inhibitor Deficiency, and Angiotensin-Converting Enzyme Inhibitor–Associated Angioedema Guideline Summary

Design and created by Guideline Central in participation with the American Academy of Allergy, Asthma & Immunology and American College of Allergy, Asthma, and Immunology.

Document Overview

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

Authoring Societies

American College of Allergy, Asthma, and Immunology

American Academy of Allergy, Asthma & Immunology

Document Publication Date: Jun 1, 2013

Page Last Reviewed/Updated: Feb 5, 2026

Document Type: Guideline

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: www.jaci-inpractice.org/article/S2213-2198(20)30878-3/fulltext

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.

Supplemental Implementation Resources

Document Scope, Criteria, and Use Cases

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).

Scope: Diagnosis, Management, Prevention, Treatment

Diseases/Conditions (MeSH)

D054179 - Angioedemas, Hereditary

D056828 - Hereditary Angioedema Type III

D056829 - Hereditary Angioedema Types I and II

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

Target Patient Population: patients with hereditary angioedema (HAE)

Health Care Settings: Ambulatory, Outpatient

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

Number of Source Documents: 175

Includes peer/external review process?: Yes

Includes public comment process?: No

Methodologist involvement?: Yes

Patient involvement?: No

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: Yes

Grades quality of strength of recommendation?: Yes

Discloses funding source?: Yes

Discloses conflicts of interest?: Yes

Includes benefits/harms analysis with recommendations?: No