What is primary immunodeficiency?

Primary immunodeficiency occurs when part of a person’s immune system is missing or does not work correctly. The bodies of people with primary immunodeficiency can’t get rid of germs or protect themselves from new germs as well as they should.

Primary immunodeficiencies are inherited, meaning they are passed down from parents to children.

These immunodeficiencies range from very serious to very mild. The serious forms are often noticeable at birth or shortly thereafter. However, very mild forms may not be diagnosed until later in life, during adolescence or young adulthood.

The World Health Organization has identified more than 80 kinds of primary immunodeficiency diseases. But, the general category includes more than 100 diseases caused by an immune system that doesn’t function properly.

What are the symptoms of primary immunodeficiency?

The most common problem for people with primary immunodeficiency disease is that they are more likely to get infections than other people are. Other symptoms include:

• Having infections more often and getting infections that are more severe, longer lasting, and harder to cure than for people with normal immune systems.
• Getting infected with germs that a healthy immune system would be able to get rid of, known as opportunistic infections.
• Having autoimmune problems, meaning that instead of the immune system attacking germs and disease-causing materials, it attacks the body’s own organs and tissues by mistake.

How is primary immunodeficiency treated?

Treatment for primary immunodeficiency falls into three stages:

1. Clear up the current infection.
2. Avoid germs and prevent exposure to new infections.
3. Correct the immunodeficiency.

Even though no one treatment works in all cases of primary immunodeficiency, there are many treatment options that can even be combined to make it more likely that primary immunodeficiency can be treated.