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Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry

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Abstract

Introduction

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.

Methods

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).

Results

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.

Conclusion

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.

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Abbreviations

PID:

Primary immune deficiency disorder

PAD:

primary antibody deficiency

QoL:

quality of life

USIDnet:

US Immunodeficiency Network

CVID:

common variable immunodeficiency

DGS:

DiGeorge syndrome

MAS:

miscellaneous antibody deficiency

AGAMMA:

agammaglobulinemia

CGD:

chronic granulomatous disease

SCID:

severe combined immunodeficiency disorder

ALPS:

autoimmune lymphoproliferative syndrome

APCED:

autoimmune polyendocrinopathy with candidiasis and ectodermal dystrophy

IPEX:

immune dysregulation, polyendocrinopathy, enteropathy X-linked

HIGM:

hyperIgM syndrome

ALC:

Absolute lymphocyte count

IgG:

immunoglobulin G

IVIG:

intravenous immunoglobulin G

SCIG:

subcutaneous immunoglobulin G

IBD:

inflammatory bowel disease

IDF:

immune deficiency foundation

TNF:

tumor necrosis factor

STAT:

signal transducer and activator of transcription

BMI:

body mass index

CFS:

chronic fatigue syndrome

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Acknowledgments

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.

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Authors and Affiliations

  1. Section of Immunology, Allergy and Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX, USA

    Joud Hajjar & Jordan S. Orange

  2. Texas Children’s Hospital, Houston, TX, USA

    Joud Hajjar & Jordan S. Orange

  3. Dan L. Duncan Institute for Clinical and Translational Research, Baylor College of Medicine, Houston, TX, USA

    Danielle Guffey & Charles G. Minard

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  1. Joud Hajjar
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  2. Danielle Guffey
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  4. Jordan S. Orange
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Correspondence to Joud Hajjar.

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Hajjar, J., Guffey, D., Minard, C.G. et al. Increased Incidence of Fatigue in Patients with Primary Immunodeficiency Disorders: Prevalence and Associations Within the US Immunodeficiency Network Registry. J Clin Immunol 37, 153–165 (2017). https://doi.org/10.1007/s10875-016-0367-1

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  • DOI: https://doi.org/10.1007/s10875-016-0367-1

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