Schmidt's Syndrome

Posted by Elaine Moore Jul 21, 2007

Schmidt’s syndrome is an autoimmune polyglandular syndrome first used to describe patients with both Addison’s disease and Hashimoto’s thyroiditis (autoimmune hypothyroidism). Today, the term Schmidt’s syndrome is used synonymously with autoimmune polyglandular syndrome type II to describe conditions of adrenal insufficiency occurring in conjunction with one or more of the following conditions: type 1 diabetes, Hashimoto’s thyroiditis, hypoparathyroidism (parathyroid hormone deficiency), or gonadal failure.

Who is Affected?

Although people of any age may be affected, Schmidt’s syndrome typically has an adult onset, primarily emerging at around age 30. Females are affected more often than males. Researchers suspect that genetic susceptibility, primarily an association with HLA B8, is responsible for the development of Schmidt’s syndrome. It’s also not unusual for more than one immediate family member to be affected.

Symptoms

Because different glands are affected in different people, the symptoms vary depending on the glands affected and the severity of the hormone deficiency or excess and the degree of glandular atrophy. Some patients with Schmidt’s syndrome have stimulating TSH receptor antibodies(also known as thyroid stimulating immunoglobulins or TSI) and initially present with symptoms and signs of hyperthyroidism.

Related Conditions

People with Schmidt’s syndrome may also develop vitiligo, type 2 diabetes, myasthenia gravis, pure red cell aplasia, pernicious anemia, Graves’ disease, alopecia, sarcoidosis, or immunoglobulin A deficiency, which is commonly seen in celiac disease.

Schmidt syndrome can also be associated with interstitial myositis, an inflammatory myopathy, which can be pathologically distinguished from idiopathic polymyositis and inclusion body myositis (IBM).

Diagnosis

Diagnosis is made by detecting adrenal antibodies along with the characteristic autoantibodies and/or hormone levels associated with autoimmune thyroid disease, diabetes, hypoarathyroidism or gonadal failure.

Resources:

Barbara Majeroni and Parag Patel, Autoimmune Polyglandular Syndrome, Type II, American Family Physician 2007; 75: 667-670.