Journal of Clinical Immunology

, Volume 37, Issue 2, pp 153–165 | Cite as

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

  • Joud Hajjar
  • Danielle Guffey
  • Charles G. Minard
  • Jordan S. Orange
Original Article

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.

Keywords

Fatigue Primary immune deficiency disorder USIDnet 

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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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Section of Immunology, Allergy and Rheumatology, Department of PediatricsBaylor College of MedicineHoustonUSA
  2. 2.Texas Children’s HospitalHoustonUSA
  3. 3.Dan L. Duncan Institute for Clinical and Translational ResearchBaylor College of MedicineHoustonUSA

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