Polyglandular autoimmune syndrome (PGA) is a rare condition that affects the body’s ability to produce hormones and other substances necessary for proper functioning. It is caused by an autoimmune disorder, meaning that the body’s immune system mistakenly attacks its own tissues. Diagnosing PGA can be difficult due to its rarity and the fact that it can manifest itself in various ways.
The diagnostic criteria for PGA is established by the American College of Rheumatology (ACR) and include:
1. The presence of two or more autoimmune diseases (or endocrinopathies) that are clinically active and affect at least two endocrine glands.
2. A family history of PGA.
3. The presence of symptoms or signs of autoimmune disease in at least two endocrine glands.
4. The absence of other causes of endocrinopathy.
5. The presence of autoantibodies in at least two endocrine glands.
6. The presence of laboratory evidence of adrenal insufficiency.
7. The presence of a serum antinuclear antibody (ANA) titer of 1:160 or higher.
8. The presence of autoantibodies to thyroid peroxidase or thyroglobulin.
In order to meet the criteria for a diagnosis of PGA, a patient must have at least two of the criteria listed above. However, the presence of all eight criteria does not guarantee a diagnosis, as the presence of other factors may influence the diagnosis. It is important to note that a diagnosis of PGA requires a comprehensive evaluation of the patient’s medical history, physical exam, and laboratory tests.
The diagnosis of PGA can help to identify potential complications of the condition, such as adrenal insufficiency, hypoglycemia, and hypothyroidism. Additionally, it can help to guide the appropriate management of the condition, including the use of immunosuppressive medications and other therapies to control the symptoms.