Primary Immunodeficiency (PI)


This is the name given to a large group of rare, chronic genetic disorders. These are diseases of the immune system. A person who has a PI has a weaker-than-normal immune system. There are hundreds of primary immunodeficiency diseases. Some are serious, and some are mild.


PI diseases are usually inherited. They result from altered or mutated genes that are passed from a parent to a child. Some develop when genes are damaged during cell division.


A common symptom of many primary immunodeficiency diseases is frequent infections. These infections can occur throughout the body. They can last a long time and can be difficult to treat. PI diseases can also cause blood disorders, digestive problems and delayed growth. And, they can lead to autoimmune disorders. Symptoms can vary greatly from person to person depending on the disease.


PI diseases are treated with various medications and therapies. These help fight existing infections. And they boost the immune system to prevent new infections. A stem cell transplant may provide a cure for some types of PI diseases.