Type II Polyglandular Autoimmune Syndrome Follow-up

Updated: Mar 26, 2018
  • Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Follow-up

Further Inpatient Care

Continuously screen patients who have had fewer than all 3 diseases every 1-2 years, until they are aged 50 years. This detects new disorders before overt clinical features develop. Screening should include an assessment of autoantibodies, electrolytes, thyroid function tests, liver function tests, vitamin B-12 levels, Cortrosyn-stimulation test, fasting blood glucose, plasma renin activity, CBCs, gonadotropins, and testosterone/estradiol. In females who have regular menses, gonadotropins and estradiol are not necessary.

Evaluate patients for asplenia, and administer pneumococcal and flu vaccinations.

Family members should be strongly considered for genetic counseling and should undergo necessary screening for autoimmune diseases.

All patients with adrenal insufficiency should wear emergency identification bracelets, because adrenal crises are a significant cause of preventable mortality in these individuals. Bracelets should indicate whether the patient also has diabetes, because the coexistence of adrenal failure increases the risk of hypoglycemia.

Patients committed to the lifelong use of minerals, vitamins, blood work, and hormonal replacement therapy require psychosocial support.

The mortality and morbidity rates associated with polyglandular autoimmune syndrome type II (PGA-II) are assumed to be identical to those of the component diseases when these disorders occur in isolation.

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Inpatient & Outpatient Medications

Administer specific hormone replacement as necessary (eg, T4, corticosteroids, sex steroids, insulin), depending on which endocrine end-organ failures have occurred.

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Complications

Complications are related to the underlying endocrine organ failure, ie, complications of diabetes in autoimmune insulitis/diabetes.

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Prognosis

See Further Inpatient Care.

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Patient Education

If autoimmune destruction of the pancreas occurs, provide extensive diabetes education for the patient. The same is true for the thyroid and other aspects.

The genetic predisposition of polyglandular autoimmune syndrome type II (PGA-II) requires educating other family members regarding testing.

For patient education resources, see the Endocrine System Center; Diabetes Center; Esophagus, Stomach, and Intestine Center; and Blood and Lymphatic System Center, as well as Thyroid Problems, Diabetes, Celiac Sprue, and Anemia.

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