Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2017 Dec 4:4:212.
doi: 10.3389/fmed.2017.00212. eCollection 2017.

Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema

Anette Bygum  1 , Paula Busse  2 , Teresa Caballero  3 , Marcus Maurer  4
Affiliations
Review

Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema

Anette Bygum et al. Front Med (Lausanne). .

Abstract

Hereditary angioedema (HAE) is a group of rare, potentially life-threatening, and frequently debilitating diseases characterized by recurrent, and often with an unpredictable onset, of swelling attacks. HAE is heterogeneous, with considerable differences between its subtypes, patients, and even within the same patient over time. During the past few years, several new on demand and prophylactic therapies have become available for HAE, allowing for individualized treatment. Therefore, to optimize HAE management, it is important to determine in all patients, the severity of their attacks, their disease activity, its therapeutic control, and its impact on their quality of life. In this manuscript, we review the existing tools to assess these aspects of HAE management, many of which are patient-reported outcome instruments. Also, we outline the current gaps of knowledge and what tools are still missing to allow for a comprehensive assessment of all patients with HAE including children.

Keywords: activity; burden; control; hereditary angioedema; impact; patient-reported outcomes; quality of life; severity.

PubMed Disclaimer

References

    1. Bork K, Barnstedt SE. Laryngeal edema and death from asphyxiation after tooth extraction in four patients with hereditary angioedema. J Am Dent Assoc (2003) 134:1088–94.10.14219/jada.archive.2003.0323 - DOI - PubMed
    1. Bork K, Maurer M, Bas M, Hartmann K, Biedermann T, Kreuz W, et al. Hereditäres angioödem durch C1-inhibitor-mangel. Allergo J (2012) 2:9.
    1. Speletas M, Szilagyi A, Psarros F, Moldovan D, Magerl M, Kompoti M, et al. Hereditary angioedema: molecular and clinical differences among European populations. J Allergy Clin Immunol (2015) 135:570–3.10.1016/j.jaci.2014.08.007 - DOI - PubMed
    1. Gomez-Traseira C, Perez-Fernandez E, Lopez-Serrano MC, Garcia-Ara MC, Pedrosa M, Lopez-Trascasa M, et al. Clinical pattern and acute and long-term management of hereditary angioedema due to C1-esterase inhibitor deficiency. J Investig Allergol Clin Immunol (2015) 25:358–64. - PubMed
    1. Roche O, Blanch A, Caballero T, Sastre N, Callejo D, Lopez-Trascasa M. Hereditary angioedema due to C1 inhibitor deficiency: patient registry and approach to the prevalence in Spain. Ann Allergy Asthma Immunol (2005) 94:498–503.10.1016/S1081-1206(10)61121-0 - DOI - PubMed

LinkOut - more resources