• Ningkun Xiao, Xinlin Huang, Dandan Yang, Wanli Zang, Sergey Kiselev, Mikhail A Bolkov, Khyber Shinwari, Irina Tuzankina, and Valery Chereshnev.
• Department of Immunochemistry, Institution of Chemical Engineering, Ural Federal University, Yekaterinburg, Russia; Laboratory for Brain and Neurocognitive Development, Department of Psychology, Institution of Humanities, Ural Federal University, Yekaterinburg, Russia. Electronic address: Fellones.zero@gmail.com.
• Prev Med. 2024 Sep 1; 186: 108079108079.

BackgroundInborn Errors of Immunity (IEI) significantly affect patients' health-related quality of life (HRQOL), presenting greater challenges than those faced by the healthy population and other chronic disease sufferers. Current research lacks comprehensive integration of this critical issue.ObjectiveThis study explores HRQOL in IEI patients, identifies impacting factors, and advocates for increased research focus on their quality of life.MethodsFollowing systematic review and meta-analysis guidelines, a search of Scopus and PubMed until November 15, 2023, yielded 1633 publications. We evaluated the literature, assessed study quality, and compared the HRQOL of IEI patients to that of healthy individuals and other chronic disease patients.ResultsOf 90 articles and 10,971 IEI patients analyzed, study quality varied (nine good, 63 moderate, and 18 poor). The Short Form-36 (SF-36) and Pediatric Quality of Life Inventory generic core scales (PedsQL) were the primary generic instruments used among adults and children, respectively, with 12 studies each using the disease-specific instruments. Meta-analysis showed IEI patients have significantly lower scores in general health, physical and mental health, and social and emotional roles compared to healthy populations. We noted significant differences between self and proxy reports, indicating caregiver anxiety and perception disparities.ConclusionDespite limitations like small sample sizes and reliance on generic instruments, this research underscores the substantially lower HRQOL among IEI patients, emphasizing the need for a patient-centered, multidisciplinary approach to improve their life quality and calling for more focused attention on IEI patients and their caregivers' HRQOL.Copyright © 2024 Elsevier Inc. All rights reserved.

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