Polyglandular Autoimmune Syndrome

The endocrine system is similar to a control panel that regulates many of our bodily functions. It consists of a group of glands and organs that produce and release hormones. Glands of the endocrine system include the pancreas, ovaries, testes, thyroid and the adrenal glands.

Polyglandular autoimmune syndromes (PAS) are a group of disorders characterized by endocrine tissue destruction, which causes multiple glands to malfunction. PAS occur when the immune system attacks the endocrine glands and cripples hormone production. Genetics also play a role in determining who develops PAS.

Types of PAS: There are four different kinds of PAS. They are distinguished by which endocrine failures are present when a diagnosis is made.

• Type 1 PAS usually affects children around the age of 10 to 12. It is a rare genetic condition that usually starts with chronic yeast infections and progresses to include the parathyroid and adrenal glands, resulting in hypoparathyroidsim and Addison's disease. Other related autoimmune disorders can develop over the years.

• Type 2 PAS occurs in adults, with women being three times more likely than men to develop it. It usually starts with adrenal gland insufficiency and progresses to include the thyroid (hypothyroidism) and the pancreas (Type 1 diabetes). Pernicious anemia also frequently accompanies PAS 2, as well as other disorders such as vitiligo, alopecia, myasthenia gravis, rheumatoid arthritis and several other autoimmune conditions.

• Type 3 PAS also mainly affects women in their 30's. It typically starts with thyroid dysfunction and progresses to other autoimmune disorders including diabetes, pernicious anemia, celiac disease and many of the same conditions that occur in PAS 1 and 2. It doesn't typically include adrenal gland malfunction.

• People with Type 4 PAS usually have two or more autoimmune endocrine diseases that don't follow the typical pattern for the other types of PAS. For instance, a person may have type 1 diabetes first and then go on to develop celiac disease without adrenal or thyroid involvement.

Diagnosis of PAS: What type of PAS is diagnosed depends on which autoimmune disorder presents itself first.

• PAS 1 usually begins with chronic yeast infections and then progresses to the parathyroid glands and then the adrenals. Other conditions may also show up along the way, including disorders of the reproductive glands.
• PAS 2 involves the adrenal glands, the thyroid and the pancreas, resulting in adrenal insufficiency, thyroiditis and type 1 diabetes.
• In PAS 3 Thyroiditis usually occurs first, followed by other autoimmune diseases, including type 1 diabetes but excluding adrenal involvement.
• PAS 4 is more likely to be diagnosed based on which endocrine glands are not affected, rather than by which ones are.

Treatments for PAS: Treatment usually is determined by which endocrine glands are affected and in which hormones the person is deficient. For example, someone with type 1 diabetes will need to take insulin, and a person with adrenal insufficiency may need to replace cortisol. Follow-up and screening for additional endocrine gland failures is necessary once PAS is diagnosed, because so many different conditions may occur later on.

Sources:

Tomer, MD, FACP, FACE, Yaron. "Genetic Susceptibility to Autoimmune Endocrine Disorders." InFocus Newsletter Vol. 12, No. 3. Sept. 2004. American Autoimmune Related Diseases Association. 10 Apr 2007.

Majeroni, MD, Barbara, and Patel, MBBS, Parag, "Autoimmune Polyglandular Syndrome, Type 2." American Family Physician. 1 Mar. 2007. American Academy of Family Physicians. 10 Apr 2007.

Cihakova, MD, PhD, Daniela. "Endocrine Diseases." Autoimmune Disease Research Center. John Hopkins Medical Institutions. 10 Apr 2007.