Overview
Primary immunodeficiency refers to a group of more than 400 disorders that affect your immune system. These disorders prevent your immune system from functioning properly. This can make you more susceptible to infection and certain diseases, including autoimmune diseases and cancers. Other names for primary immunodeficiency include:
- Primary immunodeficiency disease.
- Primary immunodeficiency disorder (PIDD).
- Inborn errors of immunity (IEI).
Genetic mutations (changes) cause primary immunodeficiency. They’re usually inherited, or passed down, within families, but can sometimes pop up sporadically. Providers treat primary immunodeficiency by preventing and managing infections and replacing missing or defective immune system components.
What are examples of primary immunodeficiency?
There are more than 400 types of primary immunodeficiency. These types vary in severity, which affects the age of onset (when they develop). Some types of PIDD cause problems during infancy and are discovered shortly after a baby is born. Other types are mild, and you may not receive a diagnosis until adulthood. Examples of primary immunodeficiency include:
- Common variable immunodeficiency (CVID).
- Ataxia-telangiectasia.
- Chronic granulomatous disease (CGD).
- DiGeorge syndrome.
- Hemophagocytic lymphohistiocytosis.
- Selective IgA deficiency.
- X-linked agammaglobulinemia.
Symptoms
When to see a doctor
Complications
- High blood pressure.
- Diabetes.
- Heart failure.
- Some types of heart valve disease.
Prevention
- Control high blood pressure, high cholesterol and diabetes.
- Don't smoke or use tobacco.
- Eat a diet that's low in salt and saturated fat.
- Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
- Get good sleep. Adults should aim for 7 to 9 hours daily.
- Maintain a healthy weight.
- Reduce and manage stress.
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