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Publications

Journal Article Observational Study Epublish

PubMed: 40332698 DOI: 10.1007/s10875-025-01869-z

Quality of Life in Japanese Patients with Primary Immunodeficiency Disease is Disrupted throughout the Year.

J Clin Immunol 45(1):90

Authors: Toshinao Kawai, Hirokazu Kanegane, Masataka Ishimura, Satoshi Okada, Nobuaki Okamatsu, Kaori Nakagawa, Madoka Go, Shinichi Noto

Affiliations

  • Division of Immunology, National Center for Child Health and Development, Tokyo, Japan.
  • Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU)/Institute of Science Tokyo, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.
  • Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
  • Department of Pediatrics, Graduate School of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan.
  • Medical Franchise RD, Japan Medical Office, Takeda Pharmaceutical Company Limited, Tokyo, Japan.
  • Rare Disease, Evidence Generation & Outcomes Research, Japan Medical Office, Takeda Pharmaceutical Company Limited, Tokyo, Japan.
  • Department of Rehabilitation, Niigata University of Health and Welfare, Niigata, Japan.

Abstract

Patients with primary immunodeficiency disease (PID) have an increased susceptibility to infection and may experience negative impacts on health-related quality of life (HR-QOL) and activities of daily living. This prospective observational study of patients aged ≥ 12 years with PID assessed HR-QOL, work impairment, and disease-related daily burden over a full year, with a focus on seasonal variation. The study period was from October 2021 to November 2023. Data were collected using an online system. HR-QOL was assessed using EuroQol-5 Dimensions-5 Levels (EQ-5D-5L) and the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36), work impairment with the Work Productivity and Activity Impairment (WPAI) questionnaire, and disease-related burden with a questionnaire designed for this study. In patients with PID (N = 56) and healthy volunteers (N = 43), no significant seasonal variation was observed in EQ-5D-5L, SF-36, or WPAI scores. With few exceptions, patients with PID had significantly lower EQ-5D-5L, SF-36, and WPAI scores than healthy volunteers in all seasons. In patients with PID, disease-related symptoms and limitations of daily living persisted throughout the year, regardless of season. In conclusion, patients with PID had lower quality of life and were more socially, physically, and mentally stressed in all seasons compared with healthy individuals.

MeSH

Quality Of Life, Primary Immunodeficiency Diseases, Healthy Volunteers, Work