Health-Related Quality of Life in Children and Adults with Primary Immunodeficiencies: A Systematic Review and Meta-analysis

BACKGROUND: Primary immunodeficiency disorders (PID) is a group of diseases that has been found to have an adverse impact on Quality of Life (QoL) and health related quality of life (HRQL). OBJECTIVE: To systematically assess available evidence on PID patients HRQL. METHODS: We performed a literature search of all studies reporting HRQL assessments in patients with PID published in English from inception to 11 April 2017 using MEDLINE and EMBASE. RESULTS: Out of 1699 articles, 37 met the inclusion criteria. HRQL was assessed by using a variety of generic instruments. CHQ-PF50 and SF-36 were the most frequently used (for children and adults, respectively). No PID-specific HRQL instruments were used for children. HRQL is significantly lower in PID adults (mean score difference -24.46, 95%CI, -34.57; -14.34) and children (-10.06, -12.95; -7.17) compared to the reference population and lower than in patients with other chronic conditions. There is a general agreement between child- and parent-reported data, although parents report child school-related HRQL as more impaired than children (6.19, 0.38; 11.99). Most studies were of low to moderate quality and had methodological limitations. CONCLUSION: Available evidence suggests that PID patients have a lower HRQL than healthy individuals and patients with other chronic conditions, including diabetes mellitus and juvenile idiopathic arthritis. No disease-specific instruments are available for children and few options are available for adults. This finding highlights the need for developing PID-specific instruments which would allow for a more sensitive evaluation of PID impact on patient health and psychological wellbeing, school/work and social activities.