About Primary Immune Deficiencies
What is a Primary Immune Deficiency Disease?

Primary immune deficiency diseases are disorders in which part of the body's immune system is missing or does not function properly. In contrast to secondary immune deficiency disease in which the immune system is compromised by factors outside the immune system, such as viruses or chemotherapy, the primary immune deficiency diseases are caused by intrinsic or genetic defects in the immune system.

There are a wide variety of primary immune deficiencies. The World Health Organization recognizes nearly 100 primary immune deficiency disease including X-linked Agammaglobulinemia (Bruton's Disease), Common Variable Immune Deficiency, Selective IgA Deficiency, and Severe Combined Immune Deficiency (boy-in-the-bubble disease). Some disorders, such as Selective IgA Deficiency can be quite common, occurring as often as 1/500 to 1/1000 individuals. Others, such as Severe Combined Immune Deficiency, may be as rare as one individual affected per million. Untreated primary immune deficiencies may be characterized by frequent life-threatening infections and debilitating illnesses.

Because of advances in our medical understanding and treatment of primary immune deficiency diseases, individuals who in the past would not have survived childhood are now able to live nearly normal lives. Many individuals affected by primary immune deficiency diseases require life long therapies including intravenous gamma globulin infusions, antibiotic therapies, or bone marrow transplantation. The Immune Deficiency Foundation is dedicated to improving the diagnosis and treatment of primary immune deficiency diseases through research and education.

Additional Information About Primary Immune Deficiency Diseases:

Additional Resources for Primary Immune Deficiency Diseases

Immune Deficiency Foundation Releases National Statistics on Primary Immune Deficiency Diseases; Results Show Average Time from Onset of Symptoms to Diagnosis Nine Years
June 20, 2003

Primary Immune Deficiency Diseases in the United States: The First National Survey of Patients and Specialists – Immune Deficiency Foundation

Primary Immunodeficiency Diseases – Report of the World Health Organization

When the Body's Defenses Are Missing: Primary Immunodeficiencies - Booklet from the National Institute of Child Health and Human Development