Autoimmune Polyglandular Syndrome (APS) is a rare group of disorders that causes the body’s immune system to attack the glands that produce hormones. This can affect several of the body’s endocrine glands, such as the thyroid, pancreas, parathyroid, and adrenal glands, leading to a wide variety of symptoms.
The exact cause of APS is not known, but it is believed to be a combination of genetic and environmental factors. It is thought that a genetic predisposition to develop APS may be inherited and passed down through families. Additionally, environmental triggers such as certain infections, medications, or other environmental factors may also play a role in the development of APS.
APS can be further divided into two main categories: Type I and Type II. Type I APS is also known as autoimmune polyglandular syndrome type 1 or APS1. This type of APS is caused by mutations in a gene known as AIRE (Autoimmune Regulator). This gene is responsible for controlling the activity of certain immune cells and helps to prevent the body from attacking its own tissues. Individuals with APS1 typically experience symptoms such as hypoparathyroidism, adrenal insufficiency, and chronic mucocutaneous candidiasis.
Type II APS is also known as autoimmune polyglandular syndrome type 2 or APS2. This type of APS is caused by an immune system disorder known as an organ-specific autoimmune disorder. This disorder causes the body to produce antibodies that attack the cells of certain endocrine glands, such as the thyroid, pancreas, parathyroid, and adrenal glands. Individuals with APS2 typically experience symptoms such as vitiligo, alopecia, pernicious anemia, and hypoparathyroidism.
It is important to note that APS can be difficult to diagnose due to the fact that symptoms can vary from person to person. Additionally, there is no single test that can definitively diagnose APS. Therefore, diagnosis is typically based on a combination of symptoms and laboratory tests. Treatment may include medications to replace the hormones that are no longer being produced by the affected glands, as well as lifestyle modifications and immune system-modifying therapies.