Lisa Forbes Satter, MD, Associate Professor of Pediatrics at Baylor College of Medicine, explains current treatment options for patients with primary Immunodeficiency (PI).

 

PI is a heterogeneous group of inherited disorders characterized by impaired immune system function. These disorders result in increased susceptibility to infections, autoimmune diseases, and malignancies. There are over 500 different types of PIs, with each one affecting a specific component of the immune system. While some PIs are relatively mild, others can be severe and even life-threatening.

As Dr. Forbes Satter explains, a common cause of immunodeficiency is malnutrition. This can be remedied with an improved diet. A treatment currently available for more severe PIs is blood transfusions. Blood transfusions can provide persons with the antibodies necessary to combat their own deficiency. In the most extreme cases of PI, such as severe combined immunodeficiency (SCID), bone marrow transplants may be necessary.  In addition, new treatments are in development to target the pathophysiology of PIs. Dr. Forbes Satter further noted that treatment results can be dramatically improved if patients are diagnosed as quickly as possible. It can take several years for patients with PI to be properly diagnosed since the symptoms can often mimic more common infections. Patients with recurring symptoms, or symptoms that do not respond to traditional therapy, should be referred to a specialist to determine if they have a PI.

To learn more about PI and other rare immune conditions, visit checkrare.com/diseases/autoimmune-auto-inflammatory-disorders/