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

Publication date: Available online 20 February 2019

Source: The Journal of Allergy and Clinical Immunology: In Practice

Author(s): Dmitrii Peshko, Ekaterina Titova, Ilya Korsunskiy, Elena Kondrikova, Federica Pulvirenti, Isabella Quinti, Oleg Blyuss, Audrey Dunn Galvin, Daniel Munblit

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.

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