A COSMIN systematic review of instruments for evaluating health-related quality of life in people with Hereditary Angioedema.

IF 3.2 2区医学 Q1 HEALTH CARE SCIENCES & SERVICES

Health and Quality of Life Outcomes Pub Date : 2025-02-13 DOI:10.1186/s12955-025-02342-6

Irene Baroni, Giulia Paglione, Giada De Angeli, Miriam Angolani, Edward Callus, Arianna Magon, Gianluca Conte, Stefano Terzoni, Maura Lusignani, Rosario Caruso, Andrea Zanichelli

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引用次数: 0

Abstract

Background: Hereditary angioedema (HAE) adversely affects health-related quality of life (HRQoL). HAE often compromises the HRQoL due to the impact on functional capacity caused by edema, pain, other symptoms, and psychosocial factors. Patient-Reported Outcome Measures (PROMs) focus on HRQoL and are crucial tools for evaluating the burden of the disease and choosing the most appropriate interventions for this population. However, no comprehensive evaluations of the characteristics of the available measurements to assess HRQoL have been conducted for this population.

Aim: To identify, analyze, and summarize the PROMs assessing HRQoL in individuals with HAE-C1-INH, addressing the gap in standardized assessment tools.

Methods: A systematic review was conducted up to December 2023 in PubMed, Scopus, Web of Science, Embase, and CINAHL databases, following PRISMA guidelines without language or time restrictions. Psychometric properties of the identified PROMs were appraised using COSMIN standards, and evidence was synthesized using a modified GRADE approach.

Results: From seven studies, five HRQoL PROMs were identified: two generic (SF-36 and SF-36v2) and three disease-specific (HAE-QoL, HAEA-QoL, and AE-QoL). These PROMs generally lacked comprehensive content, structural and cross-cultural validation, with none meeting the criteria for measurement invariance. This limitation affects their applicability across different demographics and cultures. However, the HAE-QoL and AE-QoL instruments were recognized for having moderate quality evidence, suggesting their potential reliability and validity.

Conclusions: This systematic review provides a moderate recommendation for the use of HAE-QoL and AE-QoL in assessing HRQoL in adults with HAE. Despite identified gaps, the moderate evidence quality for these tools supports their use, pending further validation, involving younger age groups and disease-specific contents in the assessments. Developing culturally and demographically adaptable PROMs is, therefore, a priority to improve the accuracy of PROMs in this field.

Review registration number: PROSPERO registration number is CRD42023440137.

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来源期刊

Health and Quality of Life Outcomes 医学-卫生保健

CiteScore

7.30

自引率

2.80%

发文量

154

审稿时长

3-8 weeks

期刊介绍： Health and Quality of Life Outcomes is an open access, peer-reviewed, journal offering high quality articles, rapid publication and wide diffusion in the public domain. Health and Quality of Life Outcomes considers original manuscripts on the Health-Related Quality of Life (HRQOL) assessment for evaluation of medical and psychosocial interventions. It also considers approaches and studies on psychometric properties of HRQOL and patient reported outcome measures, including cultural validation of instruments if they provide information about the impact of interventions. The journal publishes study protocols and reviews summarising the present state of knowledge concerning a particular aspect of HRQOL and patient reported outcome measures. Reviews should generally follow systematic review methodology. Comments on articles and letters to the editor are welcome.