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LoVerde, Daniel DO, MS1; Files, Daniel Clark MD1; Krishnaswamy, Guha MD, FACP, FCCP, FAAAI, FACAAI1,2

doi: 10.1097/CCM.0000000000002281
Concise Definitive Review

Objectives: Angioedema is a potentially life-threatening occurrence that is encountered by critical care providers. The mechanistic understanding of angioedema syndromes has improved in recent years, and novel medications are available that improve outcomes from these syndromes. This clinically focused review will describe the underlying genetics, pathophysiology, classification and treatment of angioedema syndromes, with an emphasis on the novel pharmacologic agents that have recently become available for acute treatment.

Data Sources: A MEDLINE search was conducted with the MeSH terms angioedema, acquired angioedema, hereditary angioedema type III, and angiotensin converting enzyme inhibitor-induced angioedema.

Study Selection: Selected publications describing angioedema, clinical trials, diagnosis, management, and genetics were retrieved (reviews, guidelines, clinical trials, case series), and their bibliographies were also reviewed to identify relevant publications.

Data Extraction: Data from the relevant publications were reviewed, summarized and the information synthesized.

Data Synthesis: The data obtained were used to describe the current state of diagnosis and management of various angioedema syndromes.

Conclusions: Angioedema is a life-threatening syndrome with multiple subtypes, each with a distinct pathophysiology. We present an evidence-based approach to the diagnosis and suggested management of various subtypes of angioedema. Securing the airway remains the most important intervention, followed by administration of both established and more novel pharmacologic interventions based on disease pathology.

1Division of Pulmonary, Critical Care, Allergy and Immunology, Department of Medicine, Wake Forest Baptist Medical Center, Winston-Salem, NC.

2Division of Allergy and Clinical Immunology, Department of Medicine, W.G. (Bill) Hefner VA Medical Center, Salisbury, NC.

This work was completed at Wake Forest Baptist Medical Center.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website (

Dr. Files disclosed other support (he conducts industry sponsored critical care studies as a site Principal Investigator for the following companies: Ferring, Leading Biosciences and Nestle). His institution received funding from the Francis Family Foundation, American Thoracic Society Foundation, and National Institutes of Health. Dr. Krishnaswamy disclosed other support from CSL Behring Corporation Immunodeficiency research grant support. They make a hereditary angioedema product (purified C1 esterase inhibitor), but this did not have any impact on the current article. Dr. LoVerde has disclosed that he does not have any potential conflicts of interest.

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