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

Abstract

Primary immunodeficiency diseases (PIDs) are a group of diseases that have been found to have an adverse impact on quality of life and health-related quality of life (HRQOL). To systematically assess available evidence on the HRQOL of patients with PID. We performed a literature search of all studies reporting HRQOL assessments in patients with PID published in English from inception to April 11, 2017, using MEDLINE and EMBASE. Of 1699 articles, 37 met the inclusion criteria. HRQOL was assessed by using various generic instruments. Child Health Questionnaire - Parent Form 50 and short-form 36 were the most frequently used (for children and adults, respectively). No PID-specific HRQOL instruments were used for children. HRQOL is significantly lower in adults with PID (mean score difference, -24.46; 95% CI, -34.57 to -14.34) and children (-10.06; 95% CI, -12.95 to -7.17) compared with 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 HRQOL as more impaired than do children (6.19; 95% CI, 0.38 to 11.99). Most studies were of low to moderate quality and had methodological limitations. Available evidence suggests that patients with PID have a lower HRQOL than do 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 that would allow for a more sensitive evaluation of PID impact on patient health and psychological well-being, school/work, and social activities.