Autoimmune Polyendocrinopathy Syndrome (Type-I)

Definition

Definition of Autoimmune Polyendocrinopathy Syndrome (Type-I)
A genetic autoimmune disease with an extraordinary array of clinical features but characterized most often by at least 2 of the following 3 findings: hypoparathyroidism - underfunction of the parathyroid glands which control calcium, candidiasis (yeast infection), and adrenal insufficiency (underfunction of the adrenal gland). The autoimmune polyendocrinopathy syndrome was the first systemic (bodywide) autoimmune disease found due to a defect in a single gene.

Symptoms

Symptoms of Autoimmune Polyendocrinopathy Syndrome (Type-I)
In most cases, the signs and symptoms of autoimmune polyglandular syndrome, type 1 begin in childhood or adolescence. This condition is characterized by three specific features: mucocutaneous candidiasis, hypoparathyroidism, and Addison disease. Affected individuals typically have at least two of these features, and many have all three.

Causes

Causes of Autoimmune Polyendocrinopathy Syndrome (Type-I)
Autoimmune polyglandular syndrome, type 1 can cause a variety of additional signs and symptoms, although they occur less often. Complications of this disorder can affect the skin and nails, the gonads (ovaries and testicles), the eyes, a butterfly-shaped gland at the base of the neck called the thyroid, and the digestive system. Type 1 diabetes also occurs in some patients with this condition.

Diagnosis

Diagnosis of Autoimmune Polyendocrinopathy Syndrome (Type-I)
Consult with your doctor.

Treatment

Treatment of Autoimmune Polyendocrinopathy Syndrome (Type-I)
The treatment of the autoimmune polyendocrinopathy syndrome is directed at treating the specific problems: replacing the various hormones that are in short supply, giving insulin for the diabetes, treating the yeast infections, etc. However, there is no known cure for the autoimmune polyendocrinopathy syndrome.

Prognosis

Prognosis of Autoimmune Polyendocrinopathy Syndrome (Type-I)
The prognosis (outlook) depends on whether the critical hormone deficiencies are remedied and especially whether the infections can be successfully controlled.

Prevention

Prevention of Autoimmune Polyendocrinopathy Syndrome (Type-I)
The autoimmune polyendocrinopathy syndrome goes by a confusing array of other names including autoimmune polyglandular syndrome (APS), polyglandular autoimmune syndrome (PGA), autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), hypoadrenocorticism with hypoparathyroidism and superficial moniliasis, and polyglandular deficiency syndrome.