Prevalence and morbidity of primary immunodeficiency diseases, United States 2001-2007

J Clin Immunol. 2014 Nov;34(8):954-61. doi: 10.1007/s10875-014-0102-8. Epub 2014 Sep 26.


Purpose: Few studies have estimated population prevalence and morbidity of primary immunodeficiency diseases (PIDD). We used administrative healthcare databases to estimate the prevalence of PIDD diagnoses in the United States from 2001 to 2007.

Methods: MarketScan databases compile claims from commercial health insurance plans and Medicaid, recording individual diagnoses for outpatient encounters and hospital stays. We used a cross sectional survey to estimate prevalence of PIDD using related ICD-9 codes (279.0, 279.1, 279.2, 279.8, 279.9, 288.1 and 288.2). Persons with secondary immunodeficiency diagnoses were excluded from analysis.

Results: Between 2001 and 2007, prevalence of any PIDD diagnosis increased from 38.9 to 50.5 per 100,000 among privately insured and from 29.1 to 41.1 per 100,000 among publicly insured persons. B cell defects predominated. Prevalence was more than twice as high among Whites as among Blacks or Hispanics.

Conclusion: In this large database, we found a higher prevalence of diagnosed PIDD than has been reported previously from registries. Increased awareness may have contributed to the increasing prevalence.

Publication types

  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Databases, Factual
  • Female
  • Humans
  • Immunologic Deficiency Syndromes / epidemiology*
  • Immunologic Deficiency Syndromes / mortality
  • Immunologic Deficiency Syndromes / physiopathology
  • Infant
  • Male
  • Middle Aged
  • Prevalence
  • Racial Groups
  • Retrospective Studies
  • Risk Factors
  • United States / epidemiology