Research Information System
Asthma Allergy Immunology, vol.22, no.3, pp.285-308, 2024 (ESCI, Scopus, TRDizin)
Objective: Immunoglobulin Replacement Therapy (IgRT) via intravenous (IVIG) or subcutaneous (SCIG) routes is essential for managing a large proportion of inborn errors of immunity (IEI), offering reductions in infection rates and enhancements in HealthRelated Quality of Life (HRQoL) and treatment satisfaction (TS). The assessment of HRQoL and TS among a diverse spectrum of both pediatric and adult IgRT-receiving IEI patients currently needs to be expanded. The aim of this study was to investigate both HRQoL and treatment satisfaction with current clinical status in a heterogeneous group of patients with IEI receiving IVIG and SCIG. Materials and Methods: We conducted a cross-sectional survey targeting IEI patients on IgRT, assessing TS (TSQM-9) and HRQoL (KINDL/SF-36). The survey integrated patient and caregiver perspectives with demographic, clinical, safety, and efficacy data to identify confounders of outcomes. Results: Eighty IEI patients (ages 1-45; 55 females, 45 males) participated, with 71.2% receiving IVIG and 28.8% SCIG. HRQoL scores were significantly higher for the SCIG group compared to IVIG (p=0.006), and even more so at the 20% SCIG concentration (p=0.026). History of adverse reactions to IgRT and diagnostic delay over one year showed lower TSQM-9 scores (p=0.044 and p=0.009, respectively). Patients with comorbidities also reported lower HRQoL and TSQM-9 scores compared to their peers without comorbidities (p=0.012 and p=0.046, respectively). Conclusion: SCIG, particularly at high concentration, shows an improvement in HRQoL outcomes, whereas adverse reactions to IgRT and diagnostic delay impair TS. Detrimental effect of IEI-related comorbidities on HRQoL and TS highlighted the critical role of timely and accurate diagnosis in IEI management.