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



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.


Fatigue Primary immune deficiency disorder USIDnet 



Primary immune deficiency disorder


primary antibody deficiency


quality of life


US Immunodeficiency Network


common variable immunodeficiency


DiGeorge syndrome


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


hyperIgM syndrome


Absolute lymphocyte count


immunoglobulin G


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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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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