Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults: HAE-QoL

Nieves Prior, Eduardo Remor, Elia Pérez-Fernández, Magdalena Caminoa, Carmen Gómez-Traseira, Francisco Gayá, Anne Aabom, Werner Aberer, Stephen Betschel, Isabelle Boccon-Gibod, Laurence Bouillet, Anette Bygum, Dorottya Csuka, Henriette Farkas, Maria Gomide, Anete Grumach, Iris Leibovich, Alejandro Malbran, Dumitru Moldovan, Eniko MihalyKrystyna Obtulowicz, Cecilia Perpén, Adriane Peveling-Oberhag, Grzegorz Porebski, Celine Rayonne Chavannes, Avner Reshef, Petra Staubach, Michaela Wiednig, Teresa Caballero

Research output: Article

19 Citations (Scopus)


Background: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) may affect health-related quality of life (HRQoL). A specific HRQoL questionnaire for adult patients with C1-INH-HAE, the HAE-QoL, has recently been developed in Spain. Objective: The objective of this study was to perform a cross-cultural validation and psychometric study of the HAE-QoL in an international setting. Methods: Cross-cultural adaptation of the Spanish HAE-QoL draft version and an international rating phase with experts were performed. The resultant version of the HAE-QoL, a clinical questionnaire, and Short Form 36-item Health Survey Version 2.0 (SF-36v2) were pilot tested internationally. Item reduction was based on both descriptive and exploratory factor analysis. Psychometric properties were assessed. Results: Cross-cultural adaptation of the HAE-QoL was performed in 18 countries. The draft version of the HAE-QoL was pilot tested in 332 patients, and accurate data were obtained from 290 patients from 11 countries. The reduction process resulted in a new version with 25 items and 7 dimensions (treatment difficulties, physical functioning and health, disease-related stigma, emotional role and social functioning, concern about offspring, perceived control over illness, and mental health). Strong psychometric properties were observed (Cronbach's α 0.92; test-retest reliability 0.87). Convergent validity showed mild to moderate correlations with SF-36v2 physical and mental component summaries (0.45 and 0.64, respectively) and with SF-36v2 dimensions (P < .004). HAE-QoL scores discriminated significantly among severity groups (median: asymptomatic 133.5 vs severe 84.0; P < .001); between patients with and without long-term prophylaxis (median: 101 vs 90; P = .001); and between patients with and without psychiatric and/or psychological care (median: 74 vs 103; P ≤ .001). Conclusions: The HAE-QoL, currently available in 18 languages, showed good reliability and validity evidence.

Original languageEnglish
Pages (from-to)464-473.e4
JournalJournal of Allergy and Clinical Immunology: In Practice
Issue number3
Publication statusPublished - máj. 1 2016

ASJC Scopus subject areas

  • Immunology and Allergy

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    Prior, N., Remor, E., Pérez-Fernández, E., Caminoa, M., Gómez-Traseira, C., Gayá, F., Aabom, A., Aberer, W., Betschel, S., Boccon-Gibod, I., Bouillet, L., Bygum, A., Csuka, D., Farkas, H., Gomide, M., Grumach, A., Leibovich, I., Malbran, A., Moldovan, D., ... Caballero, T. (2016). Psychometric Field Study of Hereditary Angioedema Quality of Life Questionnaire for Adults: HAE-QoL. Journal of Allergy and Clinical Immunology: In Practice, 4(3), 464-473.e4.