Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry
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Patients with primary immunodeficiency (PID) often report fatigue, yet this symptom has not been studied in PID. Fatigue affects 6–7.5% of healthy adults. The goal of this study is to estimate the prevalence of fatigue in patients with PID and investigate its associated factors.
We analyzed 2537 PID patients registered in USIDNET to determine responses to the field “fatigue” in the core registry form. Demographics, immune phenotypes, and comorbid conditions were compared between fatigued and non-fatigued patients to identify relevant associations and potential drivers. A focused analysis was performed for patients with predominantly antibody deficiency disorders (PADs).
Fatigue was reported in 25.9% (95% CI 23.7–28.3) of PAD patients, compared to 6.4% (95% CI 4.9–8.2) of non-PAD. Patients with common variable immunodeficiency (CVID) had the highest prevalence of fatigue (p < 0.001) among all PID diagnoses. Other factors that were associated with a higher rate of fatigue among PAD patients included female sex, higher BMI, depression, bronchiectasis, and autoimmunity. Additionally, fatigued PAD patients had lower absolute lymphocyte, CD3, CD4, and CD8 counts compared to non-fatigued patients.
Our findings suggest that fatigue is overrepresented in PAD patients. Prospective studies to estimate prevalence, risk factors, and fatigue etiology in PID are warranted, so therapeutic interventions can be considered.
KeywordsFatigue Primary immune deficiency disorder USIDnet
Primary immune deficiency disorder
primary antibody deficiency
quality of life
US Immunodeficiency Network
common variable immunodeficiency
miscellaneous antibody deficiency
chronic granulomatous disease
severe combined immunodeficiency disorder
autoimmune lymphoproliferative syndrome
autoimmune polyendocrinopathy with candidiasis and ectodermal dystrophy
immune dysregulation, polyendocrinopathy, enteropathy X-linked
Absolute lymphocyte count
intravenous immunoglobulin G
subcutaneous immunoglobulin G
inflammatory bowel disease
immune deficiency foundation
tumor necrosis factor
signal transducer and activator of transcription
body mass index
chronic fatigue syndrome
We grateful acknowledge Ms. Marla Goldsmith USIDNET Registry Manager and Tara Caulder Project Director for their expertise, dedication and support through every step of this project.
Compliance with Ethical Standards
Conflict of Interest
The authors declare that they have no conflict of interest.
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