Primary immunodeficiency

Primary immunodeficiency (PI) refers to a group of more than 400 rare, chronic disorders in which part of the body's immune system is missing or functions improperly. These disorders are caused by hereditary or genetic defects, and, although some disorders present at birth or in early childhood, the disorders can affect anyone, regardless of age or gender. Some affect a single part of the immune system; others may affect one or more components of the system.

Types of Primary Immunodeficiency

There are more than 400 types of PI. Some of the most common types include:

• Selective IgA Deficiency
• Common Variable Immune Deficiency (CVID)
• X-Linked Agammaglobulinemia (XLA)
• Severe Combined Immune Deficiency (SCID)
• Chronic Granulomatous Disease (CGD)

Symptoms

The symptoms of PI can vary greatly from one person to another. However, common symptoms include:

• Frequent and recurrent pneumonia, bronchitis, sinus infections, ear infections, meningitis or skin infections
• Inflammation and infection of internal organs
• Blood disorders, such as low platelet counts or anemia
• Digestive problems, such as cramping, loss of appetite, nausea and diarrhea
• Delayed growth and development
• Autoimmune disorders, such as lupus, rheumatoid arthritis or type 1 diabetes

Diagnosis

Diagnosis of PI requires a series of tests, including blood tests, genetic tests, and tests to measure immune system function.

Treatment

Treatment for PI includes Immunoglobulin Therapy, Antibiotics, Vaccines, and in severe cases, Bone Marrow Transplant.

See Also

• Secondary Immunodeficiency
• Immunodeficiency
• Immunology

Primary immunodeficiency Resources
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