7912 - Polyglandular syndrome (multiple endocrine neoplasia, autoimmune polyglandular syndrome)

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Definition

Polyglandular syndrome (multiple endocrine neoplasia, autoimmune polyglandular syndrome) is a condition characterized by decreased function of several endocrine glands.

Etiology

An endocrine gland can be destroyed by tumor, infection, or infarction. However, more commonly, autoimmune disorders result in inflammation and either partial or total destruction of the gland. When this occurs in one gland, it is often followed by impairment of several glands. The three major types of failure are:

  • Type 1 which occurs before age 35, but is usually recognized in the first decade of life. The glands that fail most frequently are the parathyroids (hypoparathyroidism), and the cortex of the adrenal gland. After puberty, over 50% of women lose function of the sex glands; men are in the 15% category for this loss of function. Chronic yeast infections of the mucous membranes and the skin are common (mucocutaneous candidiasis).

  • Type 2 which represents adult glandular failure. The cortex of the adrenal gland, the thyroid gland, and the pancreas are involved. The glands are destroyed as a result of antibodies and autoimmune activity. Type I diabetes mellitus is the most common manifestation. Mucocutaneous candidiasis does not occur in this type.

  • Type 3 which occurs in adults, and includes at least two of the following manifestations: alopecia, vitiligo, insulin-dependent diabetes mellitus, thyroid deficiency, and pernicious anemia.

Signs & Symptoms

The signs and symptoms exhibited depend upon the gland that is involved. There is no specific order for the timing of individual gland destruction.

Tests

Testing for antibodies is done to differentiate an autoimmune cause from an infection or infarction. In multiple deficiencies, elevated plasma levels of pituitary tropic hormones indicate hypothalamic-pituitary failure.

Treatment

Each specific glandular deficiency requires individual treatment. However, complications may result from multiple glandular failure. Sex organ failure does not respond to hormone treatment. Chronic yeast infections of the mucous membranes and skin are resistant to treatment. Some patients benefit from early administration of immunosuppressive medications.

Residuals

When there is decreased function in one gland, the patient should be observed over a period of years for additional glandular deficiencies.

Special Considerations

  • When there is decreased function in one gland, the patient should be observed over a period of years for additional glandular deficiencies.

Notes

None.