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

Dmitrii Peshko; Ekaterina Kulbachinskaya; Ilya Korsunskiy; Elena Kondrikova; Federica Pulvirenti; Isabella Quinti; Oleg Blyuss; Audrey Dunn Galvin; Daniel Munblit

doi:10.1016/j.jaip.2019.02.013

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

J Allergy Clin Immunol Pract. 2019 Jul-Aug;7(6):1929-1957.e5. doi: 10.1016/j.jaip.2019.02.013. Epub 2019 Feb 20.

Authors

Dmitrii Peshko¹, Ekaterina Kulbachinskaya², Ilya Korsunskiy³, Elena Kondrikova¹, Federica Pulvirenti⁴, Isabella Quinti⁴, Oleg Blyuss⁵, Audrey Dunn Galvin⁶, Daniel Munblit⁷

Affiliations

¹ Department of Paediatrics, Sechenov University, Moscow, Russia.
² Department of Paediatrics, Sechenov University, Moscow, Russia; The Research and Clinical Institute for Pediatrics named after Academician Yuri Veltischev of the Pirogov Russian National Research Medical University, Moscow, Russia.
³ Department of Paediatrics, Sechenov University, Moscow, Russia; Moscow City Paediatric Hospital #9 named after Speransky, Moscow, Russia.
⁴ Department of Molecular Medicine, Sapienza University of Rome, Rome, Italy.
⁵ Wolfson Institute of Preventive Medicine, Queen Mary University of London, London, United Kingdom; Department of Applied Mathematics, Lobachevsky State University of Nizhny Novgorod, Nizhny Novgorod, Russia.
⁶ School of Applied Psychology, University College Cork, Cork, Ireland.
⁷ Department of Paediatrics, Sechenov University, Moscow, Russia; Department of Paediatrics, Imperial College London, London, United Kingdom; The In-VIVO Global Network, an Affiliate of the World Universities Network (WUN), New York, NY; Healthcare Department of Moscow, Moscow Research and Clinical Center for Neuropsychiatry, Moscow, Russia. Electronic address: daniel.munblit08@imperial.ac.uk.

PMID: 30797077
DOI: 10.1016/j.jaip.2019.02.013

Abstract

Background: 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).

Objective: To systematically assess available evidence on the HRQOL of patients with PID.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Health-related quality of life; PID; Primary immunodeficiency diseases; Systematic review; quality of life.

Publication types

Meta-Analysis
Research Support, Non-U.S. Gov't
Systematic Review

MeSH terms

Adult
Child
Clinical Trials as Topic
Humans
Observational Studies as Topic
Primary Immunodeficiency Diseases*
Quality of Life*