About autoimmune polyglandular syndrome
Introduction
Autoimmune polyglandular syndrome is a genetic autoimmune disorder that is characterized by adrenal insufficiency and under-function of the parathyroid glands that control calcium. Autoimmune means that the immune system turns against and attacks tissues such as the liver, skin, lungs and joints and ends up crippling the hormone production. Auto immune polyglandular syndrome is an inherited recessive trait meaning that a child receives two mutant genes one from each parent. The gene responsible fro autoimmune polyglandular syndrome is known as autoimmune regulator(AIRE). Changes in this gene result in an autoimmune disorder.
Types of autoimmune polyglandular syndrome
There are four main types of autoimmune polyglandular syndrome. They are as follows;
Type 1 APS: affects young children at around the age of ten to twelve years. It is a rare genetic disorder that begins with chronic yeast infection and later progresses to the parathyroid and adrenal glands. These glands produce a hormone that regulates the use of calcium and phosphorous in the body. Malfunctioning of these glands causes a tingling sensation in the toes, fingers and lips. Fatigue, muscle pain, cramping and general body weakness are also experienced. This progress results in Addison's and hypoparathyroidsim diseases.
Type 2 APS: Affects adults and women tend to be more at risk of developing this disorder than men. It begins with adrenal insufficiency and later progresses to the pancreas and thyroid. People suffering from type 2 autoimmune polyglandular syndrome are at a high risk of developing pernicious anemia and other disorders like; alopecia, vitiligo, rheumatoid arthritis and myasthenia gravis.
Type 3 APS: affects women in their 30's. Presents itself as a thyroid dysfunction before advancing to other disorders like, pernicious anemia, celiac disease and diabetes. This polyglandular syndrome does not always include adrenal gland malfunction.
Type 4 APS: this polyglandular syndrome is characterized by two or more autoimmune endocrine diseases that have no typical pattern of presenting themselves like in the other types above.
Symptoms of autoimmune polyglandular syndrome
The symptoms developed by a patient depends on the type of autoimmune polyglandular syndrome the patient has. Polyglandular syndrome is diagnosed depending on the autoimmune disorder that occurs first.
In type 1, chronic yeast infections occur first then parathyroid glands and adrenals are affected. Later on, other disorders affecting places like the reproductive glands may occur. In type 2, the pancreas, adrenal glands and the thyroid are affected and end up resulting to adrenal insufficiency, type 1 diabetes and thyroiditis. In type 3, thyroditis occurs first and is later followed by other autoimmune disorders that include type 1 diabetes. Type 3 does not affect the adrenal glands. As for type 4, diagnosis can be made by identifying the endocrine glands that are not affected since it has no specific order of presenting itself.
Treatment of polyglandular syndrome
There is no definite cure for polyglandular syndrome. Therefore, the treatment of autoimmune polyglandular syndrome is determined by the endocrine glands and hormones that are affected and concentrates on the specific problems that affect an individual. These treatments include treating the yeast infections that occur, giving insulin to the diabetic patients and replacing the hormones that are in short supply. The diagnosis and treatment depends on whether critical hormone deficiencies are remedied or whether the occurring infections can be controlled successfully.
