Neural-immune-cardiovascular axis: from mechanistic crosstalk to therapeutic targets in cardiovascular disease.

Inborn errors of immunity (IEIs), formerly referred to as primary immunodeficiencies (PIDs), are a large heterogenous class of over 550 genetic disorders characterized by reduced or absent function in one or more components of the immune system (, ). Over 6 million adults and children globally () are estimated to be affected by IEIs, impacting approximately 1 in 1200 live births (). Patients with IEIs often experience chronic or recurrent infectious and non-infectious manifestations, and often require life-long treatment, which can include immunoglobulin replacement therapy (subcutaneous or intravenous), antimicrobial prophylaxis, hematopoietic stem cell transplantation, gene therapy, and other targeted immunomodulators (, ). Clinicians often leverage clinical practice guidelines to engage patients during the shared decision-making process, however, existing guidelines for the management of IEIs (, –) have only informally considered patient and caregiver values and preferences, without explicitly incorporating patient and caregiver perspectives towards IEI care and the aforementioned treatments. Understanding patient and caregiver values and preferences in IEI management can improve the rigor and applicability of clinical practice guidelines and future research. Thus, we conducted a systematic review to summarize and synthesize the values and preferences of patients with IEIs and their caregivers towards the management of IEIs. We conducted this review following similar methods to previous systematic reviews of patient values and preferences (–), as described below, and we report our findings in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement () (see for the completed PRISMA reporting checklist). We prospectively registered our review protocol on PROSPERO (CRD420251114032).