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PROMIS-29 survey confirms major impact of fatigue on health-related quality of life in common variable immunodeficiency

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Abstract

Health-related quality of life (HRQOL) is an emerging topic of interest in patients with immunodeficiency. Information about HRQOL in common variable immunodeficiency (CVID) is limited. The primary objective was to compare primary immunodeficiency disease (PIDD) patients with and without common variable immunodeficiency (CVID) on HRQOL domains using Patient-Reported Outcomes Measurement Information System (PROMIS-29) survey data from the United States Immunodeficiency Network (USIDNET) registry. The primary endpoint variables were scores on 7 HRQOL domains. The USIDNET registry was used to select patients with baseline PROMIS-29 data collected between 2015 and 2018. Descriptive statistics, Fisher’s exact test, and Student’s two-sample t test were used to compare patients with CVID versus patients with non-CVID on demographic and clinical characteristics. The single-sample t test was used to compare sample means to the normed population mean of 50. A general linear model approach to multiple regression with backward selection was used to remove factors that did not contribute significant information to the multivariable models, while controlling for multiple testing. Potential explanatory variables included group (CVID/non-CVID), sex, age, and BMI. Among 184 PIDD patients, 146 (79%) were diagnosed with CVID. Patients had a mean (SD) age of 53 (13.8), were predominantly female (83%), and were Caucasian (98%). PROMIS-29 results revealed a significant effect of group (CVID/non-CVID) on the anxiety, fatigue, and social participation domains, with fatigue being the most statistically significant. Fatigue, anxiety, and social participation may be key factors influencing HRQOL among patients with CVID. Future prospective longitudinal studies using PROMIS-29 will be needed to confirm these findings and to determine the mechanisms through which these factors develop in CVID, and how they can be improved.

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Abbreviations

HRQOL:

Health-related quality of life

CVID:

Common variable immunodeficiency

PROMIS-29:

Patient-Reported Outcomes Measurement Information System

PIDD:

Primary immunodeficiency diseases

USIDNET:

The US Immunodeficiency Network

IDF:

Immune Deficiency Foundation

IGRT:

Immunoglobulin replacement therapy

IRB:

Institutional Review Board

BMI:

Body mass index

IVIG:

Intravenous IgG

SCIG:

Subcutaneous IgG

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Acknowledgments

We would like to acknowledge The US Immunodeficiency Network (USIDNET), a program of the Immune Deficiency Foundation (IDF), for their help in query submission and data analysis. The USIDNET is supported by a cooperative agreement, U24AI86837, from the National Institute of Allergy and Infectious Diseases (NIAID).

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Authors and Affiliations

  1. Division of Allergy and Immunology, Icahn School of Medicine at Mount Sinai, New York, NY, USA

    Shouling Zhang

  2. Department of Pediatrics, Cohen Children’s Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New Hyde Park, NY, USA

    Shouling Zhang

  3. Biostatistics Unit, Feinstein Institutes for Medical Research, Manhasset, NY, USA

    Myriam Kline

  4. Division of Allergy and Immunology, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Ramsay L. Fuleihan

  5. Division of Pediatric Allergy, Immunology and Rheumatology, Columbia University Medical Center NewYork-Presbyterian Morgan Stanley Children’s Hospital, New York, NY, USA

    Ramsay L. Fuleihan

  6. USIDNET Consortium, Immune Deficiency Foundation, Towson, MD, USA

    USIDNET Consortium

  7. Immune Deficiency Foundation, Towson, MD, USA

    Christopher Scalchunes

  8. Division of Allergy and Immunology, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Kathleen E. Sullivan

  9. Division of Allergy and Immunology, Department of Pediatrics, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 865 Northern Boulevard, Suite 101, Great Neck, NY, 11021, USA

    Artemio M. Jongco III

  10. Center for Health Innovations and Outcomes Research, Feinstein Institute for Medical Research, Manhasset, NY, USA

    Artemio M. Jongco III

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  1. Shouling Zhang
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  2. Myriam Kline
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  4. USIDNET Consortium
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  5. Christopher Scalchunes
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  6. Kathleen E. Sullivan
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  7. Artemio M. Jongco III
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Correspondence to Artemio M. Jongco III.

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Zhang, S., Kline, M., Fuleihan, R.L. et al. PROMIS-29 survey confirms major impact of fatigue on health-related quality of life in common variable immunodeficiency. Immunol Res 68, 379–388 (2020). https://doi.org/10.1007/s12026-020-09162-4

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  • DOI: https://doi.org/10.1007/s12026-020-09162-4

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