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Forjaz MJ, Ayala A, Caminoa M, et al. HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency. J Investig Allergol Clin Immunol. 2020;31(3):246-252doi: 10.18176/jiaci.0479.
Forjaz, M. J., Ayala, A., Caminoa, M., Prior, N., Pérez-Fernández, E., Caballero, T. (2021). HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency. Journal of investigational allergology & clinical immunology, 31(3), 246-252. https://doi.org/10.18176/jiaci.0479
Forjaz, M J, et al. "HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency." Journal of investigational allergology & clinical immunology vol. 31,3 (2021): 246-252. doi: https://doi.org/10.18176/jiaci.0479
Forjaz MJ, Ayala A, Caminoa M, Prior N, Pérez-Fernández E, Caballero T. HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency. J Investig Allergol Clin Immunol. 2021 Jun 22;31(3):246-252. doi: 10.18176/jiaci.0479. Epub 2020 Jan 14. PMID: 31932270.
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J Investig Allergol Clin Immunol. 2021 Jun 22;31(3):246-252. doi: 10.18176/jiaci.0479. Epub 2020 Jan 14.

HAE-AS: A Specific Disease Activity Scale for Hereditary Angioedema With C1-Inhibitor Deficiency.

Journal of investigational allergology & clinical immunology

M J Forjaz, A Ayala, M Caminoa, N Prior, E Pérez-Fernández, T Caballero,

Affiliations

  1. National School of Public Health, Institute of Health Carlos III and REDISSEC, Madrid, Spain.
  2. Clinica Marazuela, Talavera de la Reina, Toledo, Spain.
  3. Hospital Universitario Severo Ochoa, Leganés, Madrid, Spain.
  4. Research Unit, Hospital Universitario Fundación Alcorcón, Madrid, Spain.
  5. Allergy Department, Hospital Universitario La Paz, Madrid, Spain.
  6. Hospital La Paz Institute for Health Research (IdiPaz), Madrid, Spain.
  7. Biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain.

PMID: 31932270 DOI: 10.18176/jiaci.0479

Abstract

BACKGROUND AND OBJECTIVE: The activity of hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) varies between patients and within individual patients. Objective: This study aims to develop a disease activity scale for C1-INH-HAE (HAE-AS) with sound measurement properties.

METHODS: Eleven countries participated in a prospective multicenter cohort study. A clinical questionnaire was self-completed by 290 adult patients with C1-INH-HAE. Patients also completed 2 quality of life scales, the SF-36v2 and the HAE-QoL. Rasch analysis and classic psychometric methods were used to preselect a series of clinical items: number of attacks by location and number of treated attacks, emergency room visits, psychological/psychiatric treatment, missed school/workdays in the previous 6 months; general health; and impairment in everyday work/activities due to pain.

RESULTS: The mean (SD) age was 41.5 (14.7; range, 18-84) years, and 69% were females. The final 12-item Rasch model showed that the HAE-AS had satisfactory reliability (person separation index, 0.748), local item independence, unidimensionality, and no item bias by age or sex. The HAE-AS provided scores in a linear measure, with a mean of 10.66 (3.92; range, 0-30). Further analysis with classic psychometric methods indicated that the HAE-AS linear measure presented moderate-to-high convergent validity with quality of life scales (SF-36v2: physical component, r=-0.33; mental component, 0.555; HAE-QoL, -0.61), and good discriminative validity by age, sex, and disease severity (P<.05).

CONCLUSIONS: The HAE-AS is a short, valid, reliable, and psychometrically sound measure of the activity of C1-INH-HAE that could prove useful for research studies.

Keywords: C1-INH-HAE; Clinical activity; Hereditary angioedema; Psychometric properties; Rasch analysis

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