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Complications Associated with Underweight Primary Immunodeficiency Patients: Prevalence and Associations Within the USIDNET Registry

Journal of Clinical Immunology volume 38, pages 283–293 (2018)Cite this article

Abstract

Purpose

The point prevalence of underweight status and obesity in primary immunodeficiency disease (PID) is unknown, despite the described associations between PID and weight loss and failure to thrive. The goal of this study is to estimate the prevalence of underweight status and obesity in PID patients and to investigate the associations between abnormal body weight and complications of PID.

Methods

Using the US Immunodeficiency Network (USIDNET), we performed a retrospective analysis of 653 pediatric (age 2 to 20 years) and 514 adult (age > 20) patient records with information on patient body mass index (BMI). Prevalence of underweight and obese status in PID patients was compared to data from the National Health and Nutrition Examination Survey (NHANES).

Results

After separating BMI data by year of entry to the database, we demonstrated that both adult and pediatric patients with PID had significantly higher prevalence of underweight patients in multiple years of analysis. Further examination of underweight patients by PID diagnosis revealed that underweight status in adults with CVID was associated with granulomatous disease as well as earlier age of CVID diagnosis. In the pediatric CVID cohort, underweight status was significantly associated with lymphopenia. Examination of obesity in pediatric and adult PID patients compared to NHANES database revealed only a single year when obesity in PID patients was significantly less prevalent. In other 2-year time intervals from 2005 to 2014, the prevalence of obesity was unchanged in children and adults.

Conclusions

These results quantify the prevalence of underweight status in PID in a North American population and demonstrate that whether as a result of weight loss or poor weight gain, underweight status is more prevalent in the PID population than in the general US population. The prevalence of obesity in PID patients was similar to that seen in the general population. This highlights the need for continued education on the association of low weight and PID.

Clinical Trial Registration

NCT01953016

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Abbreviations

PID:

Primary immunodeficiency diseases

FTT:

Failure to thrive

BMI:

Body mass index

USIDNET:

US Immunodeficiency Network

CDC:

Center for Disease Control

CVID:

Common variable immunodeficiency

SCID:

Severe combined immunodeficiency

CGD:

Chronic granulomatous disease

NHANES:

National Health and Nutrition Examination Survey

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Acknowledgments

We are grateful to primary immunodeficiency patients who shared their data for inclusion in USIDNET and to the members of USIDNET Body Weight Consortium who collected patient data for this cohort: Ramsay L. Fuleihan, Elizabeth Garabedian, Rebecca H. Buckley, Francisco A. Bonilla, Javeed Akhter, Daniel Suez, Jennifer Puck, Charolotte Cunningham-Rundles, Patricia Lugar, Niraj C. Patel, Elizabeth A. Secord, Elie Haddad, John Routes, Zuhair K. Ballas, Avni Joshi, Hans D. Ochs, Burcin Uygungil, Laurence Cheng, Vivian Hernandez-Trujillo, Leonard Calabrese, Karin Chen, Morna Dorsey, Mica Muskat, Mark Ballow, Mark R. Stein, Gary Kleiner, Warren Strober, Jim Fernandez, David Buchbinder, Heather Lehman, Sung-Yun Pai, Lisa Kobrynski, Luigi Notarangelo, Ralph Shapiro, Jason Caldwell, Kathleen Haines, Jason Raasch, Christine Seroogy, Andrea J. Apter, Melvin Berger, Patricia Costa Reis, Joseph DiBenedetto, Stewart Donn, Raif S. Geha, Christopher George, Gabriel E. Gonzalez, Richard J. Guillot, Kathleen E. Grundling, Caroline Horner, Robert Hostoffer, Peter Kim, Charles H. Kirkpatrick, Adina Knight, Roger H. Kobayashi, Peter Mustillo, Terry L. Overby, Marilyn Peitso, Robert Rabinowitz, Christopher Randolph, Robert L. Roberts, Phillip W. Smith, Bobo Tanner, James Verbsky, Martha White, Dowain Wright, Elizabeth M. Younger, and Grace Yu.

We would additionally like to thank Okan Elci for assistance with statistical analysis and Marla Goldsmith and Tara Caulder for help accessing USIDNET data.

Funding

MAR supported by USIDNET/Baxalta Grant and NIH T32-HD043021; KES is funded by The Wallace Chair of Pediatrics.

Author information

Affiliations

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

    Melanie A. Ruffner & Kathleen E. Sullivan

  2. Department of Pediatrics, Perelman School of Medicine at University of Pennsylvania, Philadelphia, PA, USA

    Melanie A. Ruffner & Kathleen E. Sullivan

Authors
  1. Melanie A. Ruffner
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USIDNET Body Weight Group

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Correspondence to Kathleen E. Sullivan.

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The authors declare that they have no conflict of interest.

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Ruffner, M.A., USIDNET Body Weight Group. & Sullivan, K.E. Complications Associated with Underweight Primary Immunodeficiency Patients: Prevalence and Associations Within the USIDNET Registry. J Clin Immunol 38, 283–293 (2018). https://doi.org/10.1007/s10875-018-0492-0

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Keywords

  • Primary immunodeficiency
  • common variable immunodeficiency
  • failure to thrive
  • obesity
  • underweight
  • National Health and Nutrition Examination Survey (NHANES)