Autoimmune polyendocrine syndrome (patient information)

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Autoimmune polyendocrine syndrome
ICD-10	E31.0
OMIM	240300 269200
DiseasesDB	29212 Template:DiseasesDB2

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Jinhui Wu, M.D.; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [2]

Overview

Autoimmune polyendocrine syndrome is a rare disease that consists of multiple endocrine gland insufficiencies. There are three autoimmune polyendocrine syndromes: type I, II and III. Type I and type II are also known as Schmidt syndrome. Type III often occurs in adults.

What are the symptoms of Autoimmune polyendocrine syndrome?

Type I: fungal infections (chronic mucocutaneous candidiasis), thyroid issues (hypoparathyroidism), autoimmune adrenal insufficency, type 1 diabetes, reproductive issues (hypogonadism), anemia, malabsorption, baldness, and vitiligo

Symptoms may appear as young as five years old (specifically with candidiasis). Hypoparathyroidism commonly appears in people younger than 10 years. Autoimmune adrenal insufficency appears in people younger than 15 years.

Type II: autoimmune disease (Addison's disease), thyroid disease, type 1 diabetes, reproductive issues (hypogonadism), digestive issues (Celiac disease) and neuromuscular disease (myasthenia gravis). Type III: digestive issues (Celiac disease and gastric carcinoid tumors), reproductive issues (hypogonadism), autoimmune disorders (sarcoidosis), and rheumatoid arthritis.

Who is at highest risk?

Genetics play a role in the likelihood someone may have Autoimmune polyendocrine syndrome.

Type I has been linked to a genetic defect in the 21st chromosome which influences immune tolerance.

Type II patients are at a higher risk when they carry a particular HLA genotype.

Type III has been linked to an X chromosome defect and is more common in males. Women, too, can be carriers and might suffer mildly from the disease.

When to seek urgent medical care?

Call your health care provider if your child has persistent yeast infections, thyroid issues, reproductive issues, or digestive issues seem unusual for his or her age.

Where to find medical care for autoimmune polyendocrine syndrome?

Directions to Hospitals Treating autoimmune polyendocrine syndrome

Treatment

Type I: Treatments focus on the three major clinical features, including fluconazole and ketoconazole, calcium and vitamin D, and steroid. The prognosis of autoimmune polyendocrine syndrome type I is variable, depending on how organs are affected and the severity of the disease.

Type II: Treatment involves drug therapy. Drugs for such signs mentioned above are the main treatment opinion, including replacement therapy with hydrocortisone and fludrocortisone, antithyroid medications, insulin, and medicines on other manifestations. Prognosis of autoimmune polyendocrine syndrome type II depends on whether endocrine end-organ failures occur or not.

Type III: Compared with type I and II, autoimmune polyendocrine syndrome type III does not involve the adrenal cortex. Studies demonstrate that autoimmunity, environmental factors and genetic factors are involved in the cause of autoimmune polyendocrine syndrome type III. The goals of treatment of autoimmune polyendocrine syndrome type III are to correct hormone deficiencies, prevent complications, and reduce morbidity. Treatments include monitoring of glandular functions for early detection of glandular failure, lifelong hormone replacement therapy for established glandular failure, and familial screening.

What to expect (Outlook/Prognosis)?

Type I: The prognosis of autoimmune polyendocrine syndrome type I is variable, depending on how organs are affected and the severity of the disease.

Type II: Prognosis of autoimmune polyendocrine syndrome type II depends on whether endocrine end-organ failures occur or not.

Type III: Prognosis of autoimmune polyendocrine syndrome type III depends on whether glandular failures occur or not.

Sources

• Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. N Engl J Med 2004;350:2068-79. PMID 15141045.