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Prevalence of Granulomas in Patients With Primary Immunodeficiency Disorders, United States: Data From National Health Care Claims and the US Immunodeficiency Network Registry

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Abstract

Purpose

Granulomas are a potentially severe condition that can last for several years in persons with primary immunodeficiency disorders (PIDD). We assessed the prevalence of granulomas in patients with PIDD.

Methods

We used the Truven Health MarketScan® 2005–2015 Commercial Claims and Encounters and 2006–2015 Medicaid databases and the US Immunodeficiency Network (USIDNET) PIDD registry (a program of the Immune Deficiency Foundation). Our study population consisted of persons age < 65 years with PIDD, defined as persons with ≥ 2 claims with a diagnostic code for PIDD in MarketScan databases, or patients enrolled in USIDNET. Granulomas were identified using diagnostic codes in MarketScan or provider report in USIDNET. We calculated annual prevalence of PIDD and of granulomas among PIDD patients.

Results

We identified 247,474 and 40,395 persons with PIDD among commercially and Medicaid-insured persons, respectively. PIDD prevalence was 6.0/10,000 in 2005 and 11.7/10,000 in 2015 among commercially insured persons and 5.5/10,000 in 2006 and 9.6/10,000 in 2015 among Medicaid-insured persons. The prevalence of granulomas among PIDD patients was 1.2 and 1.5% among commercially and Medicaid-insured persons, respectively. In USIDNET, prevalence of granulomas was 4.4% (177/4021). The proportion with granulomas was similar across age groups in MarketScan, but varied from 2 to 9% in USIDNET. The reported prevalence of granulomas differed depending on PIDD condition: 1–2% in the MarketScan data and 0–13% in USIDNET.

Conclusion

Granuloma prevalence in PIDD patients was 1–4%. Our study provides an estimate of the proportion of PIDD patients and suggests that granulomas are an uncommon occurrence among patients with PIDD.

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Abbreviations

AT:

Ataxia telangiectasia

CGD:

Chronic granulomatous disease

CVID:

Common variable immunodeficiency

IRB:

Institutional Review Board

PIDD:

Primary immune deficiency disorder

SCID:

Severe combined immunodeficiency

USIDNET:

United States Immunodeficiency Network registry

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Acknowledgements

We want to thank Manisha Patel, MD, MS, for her thoughtful review of the manuscript. We thank Mary Ann Hall, MPH, for her editorial assistance. The authors would also like to thank the patients and many physicians whose time and effort made the tools provided by USIDNET possible. USIDNET is supported by a cooperative agreement, U24AI86837, from the National Institute of Allergy and Infectious Diseases (NIAID)/NIH that has been awarded to the Immune Deficiency Foundation (IDF).

Authorship Contributions

Ms. Leung contributed to the study design, analyzed and interpreted the data, drafted the initial manuscript, and reviewed and revised the manuscript.

Dr. Sullivan contributed to the study design, interpreted the data, and critically reviewed the manuscript.

Dr. Perelygina contributed to the study design, interpreted the data, and critically reviewed the manuscript.

Dr. Icenogle contributed to the study design, interpreted the data, and critically reviewed the manuscript.

Dr. Fuleihan enrolled patients and collected data for the USIDNET registry, interpreted the data, and critically reviewed the manuscript.

Dr. Lanzieri contributed to the study design, interpreted the data, and critically reviewed the manuscript.

All coauthors have reviewed the manuscript and have contributed in a substantive and intellectual manner to the work described. All authors approve the final manuscript as submitted.

Funding Source

K.E.S. received support from NIH through the USIDNET grant for her work. No financial disclosures from other co-authors.

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Authors and Affiliations

  1. National Center for Immunization and Respiratory Diseases, Centers for Disease Control and Prevention, 1600 Clifton Rd NE, MS A-34, Atlanta, GA, 30333, USA

    Jessica Leung, Ludmila Perelygina, Joseph P. Icenogle & Tatiana M. Lanzieri

  2. Division of Allergy Immunology, Department of Pediatrics, The Children’s Hospital of Philadelphia, Philadelphia, PA, 19104, USA

    Kathleen E. Sullivan

  3. Division of Allergy and Immunology, Feinberg School of Medicine, Northwestern Medicine, Chicago, IL, 60611, USA

    Ramsay L. Fuleihan

Authors
  1. Jessica Leung
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Correspondence to Jessica Leung.

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The authors declare that they have no conflict of interest.

Disclaimer

The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention, US Department of Health and Human Services.

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Leung, J., Sullivan, K.E., Perelygina, L. et al. Prevalence of Granulomas in Patients With Primary Immunodeficiency Disorders, United States: Data From National Health Care Claims and the US Immunodeficiency Network Registry. J Clin Immunol 38, 717–726 (2018). https://doi.org/10.1007/s10875-018-0534-7

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  • DOI: https://doi.org/10.1007/s10875-018-0534-7

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