Primary Immunodeficiency Disorders
Posted Friday, June 11, 2021
There are over 400 different types of primary immunodeficiency disorders that range in severity.
01 | Introduction
Primary Immunodeficiency (PI) is a chronic disorder that suppresses the immune system. This disorder is caused by genetic or hereditary defects and may present itself at stages as early as birth. Individuals with PI generally lack certain immune components or have a malfunctioning immune system.
02 | Signs and Symptoms
Because PI weakens the immune system, it can lead to an inefficient protection against infections as well as cause general complications within the body. Common signs and symptoms of PI include:
- Recurrent, prolonged or severe infections
- Inflammation and infection of internal organs
- Digestive issues
- Blood disorders
- Delayed growth and development
- Autoimmune disorders
03 | Types
The different types of PIs can be classified into six categories depending on which immune system defense line is affected:
- B Cell deficiencies
- T Cell deficiencies
- Combination B and T cell deficiencies
- Defective phagocytes
- Complement deficiencies
- Unknown or idiopathic
04 | Treatments
While there is no cure for PI, there are treatments used to alleviate the negative effects of the disorder. Treatment for PI generally falls within two categories — managing infections caused by PI and enhancing the immune system.
Managing infections generally involves the treatment and prevention of infections using antibiotics. Over the counter medications such as ibuprofen and decongestants can be used to treat secondary symptoms of infection.
Other treatments aim to strengthen the immune system, such as immunoglobulin therapy, interferon-gamma therapy and growth factors.
For more information on primary immunodeficiency and supportive resources please visit www.primaryimmune.org.