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Prevalence of PI

A 2007 survey of 10,000 households by the Immune Deficiency Foundation (IDF) showed the prevalence of PI to be 1 in 1200, or 83 per 100,000 people in the U.S.1

Multiple Sclerosis - 85
Primary Immunodeficiency - 83*
Systemic Lupus Erythematosus - 43
Alpha-1 Antitrypsin Deficiency - 35
Cystic Fibrosis - 33

* Selective Immunoglobulin A (IgA) deficiency is one of the most common PI diseases.6 According to a survey conducted by the IDF, the prevalence of selective IgA deficiency is 22 out of 100,000.1,7 Excluding selective lgA deficiency, the prevalence of PI is 61 per 100,000.1,7

Once thought to be rare, primary immunodeficiency is now considered more common.

Types of PI

When classifying types of PI, antibody production defects comprise the most common forms.9,10

They are:
  • A heterogeneous group of disorders
  • Often characterized by impaired antibody-producing components of the immune system
  • Capable of leading to low quantity and/or quality of antibody
Some of the most common symptomatic examples of antibody production defects include:1
  • Common variable immune deficiency (CVID)
  • Agammaglobulinemia (X-linked or autosomal recessive)
  • Specific antibody deficiency (SAD)

References 1. Immune Deficiency Foundation. Primary immune deficiency diseases in America: 2007. The third national survey of patients. http://primaryimmune.org/idf-survey-research-center/idf-surveys?aid=1264&sa=1. Published May 1, 2009. Accessed May 17, 2013. 2. Noonan CW, Kathman SJ, White MC. Prevalence estimates for MS in the United States and evidence of an increasing trend for women. Neurology. 2002;58(1):136-138. 3. Helmick CG, Felson DT, Lawrence RC, et al, for the National Arthritis Data Workgroup. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part 1. Arthritis Rheum. 2008;58(1)15-25. 4. Falk JA. Diagnosis and treatment options for patients with alpha-1 antitrypsin deficiency. US Respir Dis. 2010;5(1):28-31. 5. O'Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009;373(9678):1891-1904. 6. Immune Deficiency Foundation. Selective IgA deficiency. http://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/selective-iga-deficiency/. Accessed October 23, 2013. 7. Boyle JM, Buckley RH. Population prevalence of diagnosed primary immunodeficiency diseases in the United States. J Clin Immunol. 2007;27(5):497-502. 8. Modell F, Puente D, Modell V. From genotype to phenotype. Further studies measuring the impact of a Physician Education and Public Awareness Campaign on early diagnosis and management of primary immunodeficiencies. Immunol Res. 2009;44(1-3):132-149. 9. American Academy of Allergy, Asthma & Immunology. Eight guiding principles for effective use of IVIG for patients with primary immunodeficiency. http://primaryimmune.org/wp-content/uploads/2011/11/Guiding-Principles-I.pdf. Accessed October 23, 2013. 10. Modell V, Gee B, Lewis DB, et al. Global study of primary immunodeficiency diseases (PI)-diagnosis, treatment, and economic impact: an updated report from the Jeffrey Modell Foundation. Immunol Res. 2011;51(1):61-70.

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