title: Life with a Primary Immune Deficiency: a Systematic Synthesis of the Literature and Proposed Research Agenda.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: National Institute of Allergy and Infectious Disease, NIH, 10 Center Drive, Building 10, Room 12C103, Bethesda, MD, USA. morgan.similuk@nih.gov.
authors.forename: Morgan N
authors.identifier: http://orcid.org/0000-0002-0403-2689
authors.initials: MN
authors.lastname: Similuk
authors.affiliation: Clinical Research Directorate/Clinical Monitoring Research Program, Leidos Biomedical Research, Inc., Frederick National Laboratory for Cancer Research, Frederick, MD, 21702, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Wang
authors.affiliation: National Institute of Allergy and Infectious Disease, NIH, 10 Center Drive, Building 10, Room 12C103, Bethesda, MD, USA.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Lenardo
authors.affiliation: National Human Genome Research Institute, NIH, Bethesda, MD, USA.
authors.forename: Lori H
authors.initials: LH
authors.lastname: Erby
mesh_terms: D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology* / Q000523:psychology*; D011788:Quality of Life
keywords: Quality of life; patient-centered; primary immune deficiency; psychosocial