Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies

Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies is a rare condition in which the body's immune system mistakenly produces antibodies that block interferon-gamma, a key protein that helps fight infections. Interferon-gamma is essential for activating certain white blood cells (especially macrophages) that defend against bacteria, fungi, and other organisms that live inside cells. When these antibodies neutralize interferon-gamma, the immune system becomes severely weakened, leaving patients vulnerable to unusual and hard-to-treat infections. This condition typically appears in previously healthy adults, usually between the ages of 30 and 60. Patients often develop infections caused by organisms that rarely cause disease in people with normal immune systems, known as opportunistic infections. The most common infections involve nontuberculous mycobacteria (NTM), which can affect the lymph nodes, bones, joints, lungs, and skin. Patients may also develop infections from fungi like Talaromyces (Penicillium) marneffei, Cryptococcus, Histoplasma, and other unusual organisms such as Salmonella or Burkholderia. Symptoms often include recurring fevers, swollen lymph nodes, skin lesions, bone pain, persistent cough, weight loss, and night sweats. Treatment involves long-term use of multiple antibiotics or antifungal medications to control infections. In some cases, doctors use therapies to reduce the harmful autoantibodies, such as rituximab (a medication that targets the immune cells producing the antibodies) or cyclophosphamide. Despite treatment, infections tend to recur, and managing this condition requires ongoing medical care from specialists experienced with immunodeficiency disorders.

Bring these to your next appointment

• Q1.What specific infections do I have, and what organisms are causing them?,How long will I need to take antibiotics or antifungal medications?,Am I a candidate for rituximab or other treatments to reduce my autoantibodies?,How often will I need blood tests and imaging to monitor my condition?,Are there activities or environments I should avoid to reduce my risk of new infections?,What symptoms should prompt me to seek emergency care?,Are there clinical trials or research studies I could participate in?

Common questions about Adult-onset immunodeficiency with anti-interferon-gamma autoantibodies

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