Primary Immunodeficiency

What is Primary Immunodeficiency?

Primary Immunodeficiency or PI is also called primary immunodeficiency disease or PIDD. The term defines a series of conditions and is an umbrella term for more than 300 diseases of the body’s natural self-defense or immune system.

There is no one specific PI disease. All of the diseases included in this category are considered rare and chronic by the Immune Deficiency Foundation. The good news is that none of the diseases are contagious. The bad news is that all of the disease are inherited diseases and could be passed on to children of PI patients.

What are the Symptoms of Primary Immunodeficiency?

Symptoms vary depending on which of the 300 + PI diseases a patient has. The most common symptom of all is that patients get infections more often and get infections which do not go away. People who need more than two kinds of antibiotics, including IV antibiotics, to stop an infection may have PI.

Other common symptoms include sudden loss of weight; children that grow more slowly than normal; recurrent bouts of ear infections; recurrent bouts of pneumonia; enlarged spleen; constantly swollen lymph nodes; abscesses in the skin or in the internal organs.

How is Primary Immunodeficiency Treated?

Primary immunodeficiency diseases are not curable and stay with the patient all throughout his or her life. However, many of the symptoms can be treatable so that patients can lead mostly normal lives, fulfilling commitments to family, friends and employers. Treatment depends on the symptoms, what parts of the immune system are affected and health history of the patient. One treatment plan that works for one patient may not work for another. Treatment plans can range from prescription allergy medications or injections to stem cell therapy.

One treatment that works for some patients is called immunoglobulin therapy. Blood plasma has many different kinds of antibodies or immunoglobulins. Plasma from tens of thousands of volunteers is given intravenously to the patient. The theory is that the immunoglobulins from the donors help give the patient a more normal immune system.

Last Reviewed:
October 09, 2016
Last Updated:
August 29, 2017