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Severe Fatigue Is Common Among Pediatric Patients with Primary Immunodeficiency and Is Not Related to Disease

Linde N Nijhof1, Marco van Brussel2, Esther M Pots3

  • 1Department of Pediatrics, Wilhelmina Children's Hospital, University Medical Center Utrecht, Surface mail: HP KE.04.133.1, Post box 85090, 3508 AB, Utrecht, The Netherlands. l.n.nijhof@umcutrecht.nl.

Journal of Clinical Immunology
|March 17, 2021
PubMed

Insights

Severe fatigue affects nearly 20% of pediatric patients with primary immunodeficiency (PID), impacting quality of life but not disease activity. Addressing fatigue may improve well-being in these children.

Area of Science:

  • Immunology
  • Pediatrics
  • Clinical Research

Background:

  • Fatigue is a common and distressing symptom in pediatric patients diagnosed with primary immunodeficiency (PID).
  • The specific relationship between fatigue severity and underlying disease activity in these patients remains largely unexplored.
  • Understanding this relationship is crucial for developing effective management strategies.

Purpose of the Study:

  • To investigate the prevalence of severe fatigue in pediatric patients with PID.
  • To assess the impact of fatigue on health-related quality of life (HRQoL).
  • To examine the influence of disease activity and comorbidity on fatigue severity.

Main Methods:

  • A cross-sectional study involving pediatric patients aged 2-18 years with PID.
  • Assessment of fatigue using the Pediatric Quality of Life Inventory Multidimensional Fatigue Scale (PedsQL MFS).
  • Evaluation of HRQoL using PedsQL Generic Core Scales (PedsQL GCS).
  • Statistical analyses including linear regression and ANCOVA, adjusted for age and sex, comparing with a healthy control group.

Main Results:

  • A high participation rate of 87% (79 out of 91 patients) was achieved.
  • Pediatric patients with PID exhibited significantly higher fatigue levels than healthy controls, with a severe fatigue prevalence of 18.9%.
  • Increased fatigue was inversely correlated with HRQoL across all domains and directly associated with increased school absences.
  • Severe fatigue prevalence was similar across Common Variable Immunodeficiency (CVID), Combined Immunodeficiency (CID), and Selective Immunoglobulin A Deficiency (SIgAD), but absent in the studied X-linked Agammaglobulinemia (XLA) cohort.
  • No significant association was found between fatigue severity and disease activity or comorbidity.

Conclusions:

  • Severe fatigue is prevalent in approximately 20% of pediatric PID patients, spanning various PID subtypes.
  • This fatigue significantly diminishes patients' quality of life and daily functioning.
  • Fatigue severity is independent of disease activity or comorbidities in this population.
  • Targeting fatigue presents a promising therapeutic avenue for enhancing the overall well-being and quality of life for pediatric patients with PID.
Abstract

Keywords:
Pediatricdisease activityfatiguehealth-related quality of lifeimmunodeficiency

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