Journal of Clinical Immunology

, Volume 36, Issue 2, pp 123–133 | Cite as

Life with a Primary Immune Deficiency: a Systematic Synthesis of the Literature and Proposed Research Agenda

  • Morgan N. Similuk
  • Angela Wang
  • Michael J. Lenardo
  • Lori H. Erby
Original Article



The clinical immunology literature is punctuated with research on psychosocial dimensions of illness. Studies investigating the lived experiences and stated needs of patients with primary immune deficiencies and their families are essential to improving clinical management and determining the research questions that matter to patients and other stakeholders. Yet, to move the field forward, a systematic review of literature and proposed agenda is needed.


A systematic review was conducted via PubMed and Scopus to include original research on psychological, social, or behavioral aspects of primary immune deficiencies published between 1999 and 2015. A Title/Abstract keyword search was conducted, 317 candidate article abstracts were manually reviewed, and forward/backward reference searches were completed.


Twenty-nine studies met inclusion criteria. These illuminate the complex psychological, social, and emotional experiences of primary immune deficiency. Themes included the potential for negative psychosocial impact from disease; adaptation over time; the multi-dimensional assessments of quality of life; familial impact; the important roles of hope, developing a sense of control, social support; and addressing anxiety/depression in our patients and their families. Methodological considerations and areas for improvement are discussed.


We propose the research agenda focus on study creativity and rigor, with improved engagement with existing literature and critical study design (e.g., methodology with adequate statistical power, careful variable selection, etc.). This review highlights opportunities to advance psychosocial research and bring a brighter future to clinicians, researchers, and families affected by primary immune deficiency.


Quality of life psychosocial primary immune deficiency patient-centered 



Steven M. Holland for input on draft and Joie Davis for thoughtful discussion.

This project has been funded in whole or in part with federal funds from the National Cancer Institute, National Institutes of Health, under Contract No. HHSN261200800001E. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government. This research was supported in part by the Intramural Research Program of the National Institute of Allergy and Infectious Diseases, NIH.

Compliance with Ethical Standards

Conflict of Interest



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Copyright information

© Springer Science+Business Media New York (outside the USA) 2016

Authors and Affiliations

  1. 1.National Institute of Allergy and Infectious Disease, NIHBethesdaUSA
  2. 2.Clinical Research Directorate/Clinical Monitoring Research Program, Leidos Biomedical Research, Inc.Frederick National Laboratory for Cancer ResearchFrederickUSA
  3. 3.National Human Genome Research Institute, NIHBethesdaUSA

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