Type III Polyglandular Autoimmune Syndrome Treatment & Management

Updated: Sep 22, 2017
  • Author: KoKo Aung, MD, MPH, FACP; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
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Treatment

Medical Care

Medical care of patients with polyglandular autoimmune syndrome (PAS) III includes monitoring of glandular functions for early detection of glandular failure, lifelong hormone replacement therapy for established glandular failure or failures, and familial screening. Details of different approaches of treating each component disease are beyond the scope of this article. Monitoring of glandular functions is discussed in Lab Studies.

Autoimmune thyroiditis

  • Patients with hypothyroidism need lifelong thyroxine therapy. Thyrotropin levels should be monitored by using highly sensitive assays to maintain a euthyroid state.
  • Overreplacement with thyroxine may result in osteoporosis and increased risk of atrial fibrillation.

Immune-mediated diabetes

  • The mainstay of immune-mediated diabetes (IMD) treatment is lifelong replacement therapy with exogenous insulin injections. Monitor the progress of disease by periodic retina examination, foot examination, and measurement of glycosylated hemoglobin level and the ratio of urine microalbumin to creatinine.
  • The late 20th-century development of recombinant human insulin was a major breakthrough in the treatment of IMD. Intensive insulin therapy has improved the long-term outcome of the disease at the expense of frequent hypoglycemia.
  • Pancreatic transplantation is becoming an option but usually is reserved for patients with end-stage renal disease who already require renal transplantation.
  • Specific therapies aimed at suppressing the immune response of the pancreatic islet cells are being researched.

Pernicious anemia

  • The mainstay of PA treatment is lifelong replacement therapy with parenteral hydroxocobalamin. Within 48-72 hours of the first injection, serum potassium levels drop precipitously because of rapid regeneration of red blood cells.
  • Hypokalemia may be severe enough to necessitate replacement therapy. Serum iron levels also drop precipitously for the same reason.
  • If the patient initially has a marginal iron reserve, this may halt the recovery of anemia. Some experts suggest giving a small dose of iron supplements concurrently to prevent this phenomenon.

Vitiligo

  • Psychological counseling is an essential component of treatment because the disease can negatively impact self-esteem and self-image. Many treatment options are available, but results are rather disappointing.
  • Refer to the Medscape Reference topic Vitiligo for details of different treatment approaches, which are beyond the scope of this article.

Alopecia

  • Treatment of alopecia areata depends on the patient's age and the severity of the condition.
  • Refer to the Medscape Reference topic Alopecia Areata for details of different treatment approaches, which are beyond the scope of this article.
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Consultations

Consultations include the following:

  • Endocrinologist
  • Dermatologist
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Diet

Patients with IMD require individualized dietary prescriptions to achieve therapeutic goals. Details of dietary management of IMD are beyond the scope of this article and are not discussed.

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Activity

Individuals with PAS III can continue to participate in all regular activities, although individual component glandular diseases, such as IMD, can dramatically alter a patient's life.

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