OBSOLETE: TACI-related selective deficiency of IgA

TACI-related selective deficiency of IgA is a condition in which the body does not produce enough of a specific type of antibody called immunoglobulin A (IgA). IgA is an important part of the immune system, especially for protecting the surfaces of the body that are exposed to the outside world, such as the lining of the nose, throat, lungs, and gut. This condition is linked to changes (variants) in the TNFRSF13B gene, which provides instructions for making a protein called TACI (Transmembrane Activator and CAML Interactor). TACI plays a key role in helping immune cells called B cells mature and produce antibodies. When TACI does not work properly, the body may fail to make enough IgA. Many people with selective IgA deficiency have no symptoms at all and may never know they have the condition. However, some individuals experience frequent infections, particularly of the sinuses, ears, lungs, and digestive tract. Some may also develop allergies, asthma, or autoimmune conditions where the immune system mistakenly attacks the body's own tissues. In rare cases, the condition can progress to a more serious immune deficiency called common variable immunodeficiency (CVID). Note: This entry is marked as 'OBSOLETE' in Orphanet, meaning it may have been reclassified or merged into a broader disease category. Patients should discuss with their doctor whether their diagnosis falls under selective IgA deficiency or CVID. Treatment depends on symptoms and may include antibiotics for infections, monitoring for autoimmune complications, and in some cases immunoglobulin replacement therapy if the deficiency is severe or progresses.

Bring these to your next appointment

• Q1.How severe is my IgA deficiency, and are my other immunoglobulin levels normal?,What is my risk of progressing to common variable immunodeficiency (CVID)?,Should my family members be tested for this condition?,Are there any vaccines I should avoid or take special precautions with?,What should I do if I need a blood transfusion — are there special precautions?,How often should I have my immune function monitored?,What signs should prompt me to seek urgent medical care?

Common questions about OBSOLETE: TACI-related selective deficiency of IgA