Quality of Life Evaluation in Saudi Arabian Pediatric Patients with Primary Immunodeficiency Diseases Receiving 20% Subcutaneous IgG Infusions at Home

J Clin Immunol. 2023 Aug;43(6):1360-1366. doi: 10.1007/s10875-023-01507-6. Epub 2023 May 5.

Abstract

Background and aims: Subcutaneous immunoglobulin (SCIG) home infusion is widely used as an alternative to intravenous immunoglobulin (IVIG). This study aimed to determine the quality of life (QoL) of patients with primary immunodeficiency (PID) after switching to home-based SCIG.

Methods: In this prospective open-label single-center study, QoL was determined using the validated Arabic version of the Child Health Questionnaire at baseline and 3 and 6 months after switching from IVIG to SCIG.

Results: Twenty-four patients were recruited from July 2018 to August 2021, including 14 females and 10 males. The median age of the patients was 5 years (range, 0-14 years). The patients' diagnoses included severe combined immunodeficiency, combined immunodeficiency, agammaglobulinemia, Omenn syndrome, immunodysregulation, hyper-IgE syndrome, common variable immunodeficiency, and bare lymphocyte syndrome. The median duration on IVIG before inclusion was 40 months (range, 5-125 months). The QoL score showed a significant improvement in the patients' global health at 3 and 6 months compared with those at baseline and a significant improvement in the patients' general health at 3 and 6 months compared with that at baseline. The mean baseline serum IgG trough level was 8.8 ± 2.1 g/L. The mean serum IgG level was significantly higher on SCIG at both 3 and 6 months (11.7 ± 2.3 and 11.7 ± 2.5 g/L, respectively).

Conclusions: This is the first study involving an Arab population to show improvement in the QoL of patients with PID after switching from hospital-based IVIG to home-based 20% SCIG.

Keywords: Immunoglobulin replacement therapy; hypogammaglobinemia; intravenous immunoglobulin; primary antibody deficiency; severe combined immunodeficiency.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Immunoglobulin G / therapeutic use
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Deficiency Syndromes* / drug therapy
  • Immunologic Deficiency Syndromes* / therapy
  • Infant
  • Infant, Newborn
  • Infusions, Subcutaneous
  • Male
  • Primary Immunodeficiency Diseases* / drug therapy
  • Prospective Studies
  • Quality of Life
  • Saudi Arabia

Substances

  • Immunoglobulins, Intravenous
  • Immunoglobulin G