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Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report.

Publication ,  Journal Article
Bork, K; Anderson, JT; Caballero, T; Craig, T; Johnston, DT; Li, HH; Longhurst, HJ; Radojicic, C; Riedl, MA
Published in: Allergy Asthma Clin Immunol
April 19, 2021

BACKGROUND: Hereditary angioedema (HAE) is a rare disease characterized by unpredictable, potentially life-threatening attacks, resulting in significant physical and emotional burdens for patients and families. To optimize care for patients with HAE, an individualized management plan should be considered in partnership with the physician, requiring comprehensive assessment of the patient's frequency and severity of attacks, disease burden, and therapeutic control. Although several guidelines and consensus papers have been published concerning the diagnosis and treatment of HAE, there has been limited specific clinical guidance on the assessment of disease burden and quality of life (QoL) in this patient population. Practical guidance is critical in supporting effective long-term clinical management of HAE and improving patient outcomes. The objective of this review is to provide evidence-based guidelines for an individualized assessment of disease burden and QoL in patients with HAE. METHODS: A consensus meeting was held on February 29, 2020, consisting of 9 HAE experts from the United States and Europe with extensive clinical experience in the treatment of HAE. Consensus statements were developed based on a preliminary literature review and discussions from the consensus meeting. RESULTS: Final statements reflect the consensus of the expert panel and include the assessment of attack severity, evaluation of disease burden, and long-term clinical management of HAE caused by C1-esterase inhibitor deficiency. Patient-reported outcome measures for assessing HAE attack severity and frequency are available and valuable tools; however, attack frequency and severity are insufficient markers of disease severity unless they are evaluated in the broader context of the effect on an individual patient's QoL. QoL assessments should be individualized for each patient and minimally, they should address the interference of HAE with work, school, social, family, and physical activity, along with access to and burden of HAE treatment. Advances in HAE therapies offer the opportunity for comprehensive, individualized treatment plans, allowing patients to achieve minimal attack burden with reduced disease and treatment burden. CONCLUSION: This consensus report builds on existing guidelines by expanding the assessment of disease burden and QoL measures for patients with HAE.

Duke Scholars

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Published In

Allergy Asthma Clin Immunol

DOI

ISSN

1710-1484

Publication Date

April 19, 2021

Volume

17

Issue

1

Start / End Page

40

Location

England

Related Subject Headings

  • Allergy
  • 3204 Immunology
  • 1107 Immunology
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Bork, K., Anderson, J. T., Caballero, T., Craig, T., Johnston, D. T., Li, H. H., … Riedl, M. A. (2021). Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report. Allergy Asthma Clin Immunol, 17(1), 40. https://doi.org/10.1186/s13223-021-00537-2
Bork, Konrad, John T. Anderson, Teresa Caballero, Timothy Craig, Douglas T. Johnston, H Henry Li, Hilary J. Longhurst, Cristine Radojicic, and Marc A. Riedl. “Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report.Allergy Asthma Clin Immunol 17, no. 1 (April 19, 2021): 40. https://doi.org/10.1186/s13223-021-00537-2.
Bork K, Anderson JT, Caballero T, Craig T, Johnston DT, Li HH, et al. Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report. Allergy Asthma Clin Immunol. 2021 Apr 19;17(1):40.
Bork, Konrad, et al. “Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report.Allergy Asthma Clin Immunol, vol. 17, no. 1, Apr. 2021, p. 40. Pubmed, doi:10.1186/s13223-021-00537-2.
Bork K, Anderson JT, Caballero T, Craig T, Johnston DT, Li HH, Longhurst HJ, Radojicic C, Riedl MA. Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report. Allergy Asthma Clin Immunol. 2021 Apr 19;17(1):40.

Published In

Allergy Asthma Clin Immunol

DOI

ISSN

1710-1484

Publication Date

April 19, 2021

Volume

17

Issue

1

Start / End Page

40

Location

England

Related Subject Headings

  • Allergy
  • 3204 Immunology
  • 1107 Immunology