Endocrine glands Endocrine Glands The endocrine system consists of a group of glands and organs that regulate and control various body functions by producing and secreting hormones. Hormones are chemical substances that affect... read more are organs that secrete one or more specific hormones. The actual cause of the endocrine gland malfunctioning may be related to an autoimmune reaction Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers an autoimmune disorder is not known. Symptoms vary depending... read more in which the body's immune defenses mistakenly attack the body’s own cells. Genetic factors and environmental triggers (such as viral infections, dietary factors, or certain medications) may be involved. Polyglandular deficiency syndromes are classified into three types:
Type 1: Type 1 is also called autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED). In this type, which develops in children, the parathyroid glands Overview of Parathyroid Function (See also Overview of the Thyroid Gland; Hypercalcemia; and Hypocalcemia.) The thyroid is a small gland, measuring about 2 inches (5 centimeters) across, that lies just under the skin below... read more and adrenal glands Adrenal Insufficiency In adrenal insufficiency, the adrenal glands do not produce enough adrenal hormones. Adrenal insufficiency may be caused by a disorder of the adrenal glands, a disorder of the pituitary gland... read more can be underactive. Affected people may be prone to chronic yeast infections (called chronic mucocutaneous candidiasis Chronic Mucocutaneous Candidiasis Chronic mucocutaneous candidiasis, a hereditary immunodeficiency disorder, is persistent or recurring infection with Candida (a fungus) due to malfunction of T cells (a type of white... read more ) as well. Yeast infections may be the first sign of a problem, particularly in children younger than 5 years. People may have other autoimmune disorders, including thyroid disease Overview of the Thyroid Gland The thyroid is a small gland, measuring about 2 inches (5 centimeters) across, that lies just under the skin below the Adam’s apple in the neck. The two halves (lobes) of the gland are connected... read more , diabetes mellitus Type 1 diabetes Diabetes mellitus is a disorder in which the body does not produce enough or respond normally to insulin, causing blood sugar (glucose) levels to be abnormally high. Urination and thirst are... read more , autoimmune hepatitis Overview of Hepatitis Hepatitis is inflammation of the liver. (See also Overview of Acute Viral Hepatitis and Overview of Chronic Hepatitis.) Hepatitis is common throughout the world. Hepatitis can be Acute (short-lived) read more , and certain digestive system disorders that cause difficulty absorbing nutrients (malabsorption Overview of Malabsorption Malabsorption syndrome refers to a number of disorders in which nutrients from food are not absorbed properly in the small intestine. Certain disorders, infections, and surgical procedures can... read more ).
Type 2: Type 2 is also called Schmidt syndrome. In this type, which develops in adults (particularly women), the adrenal and thyroid glands are underactive, although the thyroid gland sometimes becomes overactive. People with type 2 polyglandular deficiency may also develop diabetes Diabetes Mellitus (DM) Diabetes mellitus is a disorder in which the body does not produce enough or respond normally to insulin, causing blood sugar (glucose) levels to be abnormally high. Urination and thirst are... read more .
Type 3: This type is very similar to type 2, except that the adrenal glands remain normal.
In people with polyglandular deficiency syndromes, symptoms depend on which endocrine organs are affected. The hormone deficiencies do not always appear at the same time and may require a period of years to develop. The deficiencies do not occur in a particular sequence, and not every person will have all of the deficiencies associated with each type.
Symptoms of Polyglandular Deficiency Syndromes
In people with polyglandular deficiency syndromes, symptoms depend on which endocrine organs are affected.
Underactive adrenal gland Adrenal Insufficiency In adrenal insufficiency, the adrenal glands do not produce enough adrenal hormones. Adrenal insufficiency may be caused by a disorder of the adrenal glands, a disorder of the pituitary gland... read more : Weakness, loss of appetite, vomiting, dark skin patches, and, in severe cases, low blood pressure and death if not treated
Underactive thyroid gland Hypothyroidism Hypothyroidism is underactivity of the thyroid gland that leads to inadequate production of thyroid hormones and a slowing of vital body functions. Facial expressions become dull, the voice... read more : Weight gain, constipation, dry hair and skin, and sometimes inability to tolerate cold weather
Underactive parathyroid gland Hypoparathyroidism Hypoparathyroidism is a deficiency of parathyroid hormone (PTH) often caused by an autoimmune disorder, treatment-related damage to the parathyroid glands, or removal of the glands during surgery... read more : Tingling around the mouth, cramping of hands and feet, and seizures
Diagnosis of Polyglandular Deficiency Syndromes
Blood tests to measure hormone levels
Doctors suspect a polyglandular deficiency syndrome because of the specific symptoms. The diagnosis is confirmed by detecting deficient hormone levels in a sample of blood. Sometimes doctors also measure specific antibodies to look for an autoimmune reaction to the affected gland.
Because other endocrine organs may not malfunction for many years, doctors usually do blood tests at regular intervals in people with hormone deficiencies to ensure that any new hormone deficiency is identified as soon as possible.
Because these syndromes are often inherited, genetic testing of the affected person's relatives may be done.
Treatment of Polyglandular Deficiency Syndromes
Treatment is replacement of any hormones that are deficient. Treatment of multiple hormone deficiencies may be more complex than treating deficiency of a single hormone.
People who are prone to yeast infections may need long-term treatment with an antifungal drug.