Values and preferences of patients with inborn errors of immunity and their caregivers: a systematic review

Understanding patient values and preferences can provide valuable insight and help improve patient care for patients with inborn errors of immunity (IEIs). Therefore, this review sought to systematically review the values and preferences of patients with IEIs and their caregivers towards the management of IEIs. We searched MEDLINE, Embase, PsycINFO, and CINAHL from database inception up to June 7, 2026, for studies addressing the values and preferences of patients with IEIs or their caregivers. Paired reviewers independently screened citations, extracted data, and assessed risk of bias. We used iterative thematic content analysis to qualitatively synthesize our findings and the GRADE-CERQual approach to rate the confidence of the evidence supporting each theme. Fifty-two studies enrolled 6,388 participants. We identified 6 key themes: (1) Patients are probably more willing to engage with and accept treatment if counseled by healthcare professionals knowledgeable in IEIs (moderate confidence). Patients probably place a high value on IEI treatments that (2) minimize needle pricks and adverse effects, and (3) maximize independence, efficiency, and flexibility (moderate confidence). (4) Patients probably place a high value on effective transitions from pediatric to adult care services (moderate confidence). (5) Patients may have significant concerns about the financial impact of IEI management (low confidence). (6) Patients may place a high value on the integration of mental health and social support into their care (low confidence). Ultimately, these 6 key themes on the values and preferences of patients with IEIs and their caregivers may inform optimal clinical practice and future research in IEIs.

https://www.crd.york.ac.uk/PROSPERO/, identifier CRD420251114032.