Type III Polyglandular Autoimmune Syndrome Treatment & Management

  • Author: KoKo Aung, MD, MPH, FACP; Chief Editor: George T Griffing, MD   more...
 
Updated: Jan 3, 2012
 

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 eMedicine 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 eMedicine topic Alopecia Areata for details of different treatment approaches, which are beyond the scope of this article.
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Consultations

  • 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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Proceed to Medication
 
 
Contributor Information and Disclosures
Author

KoKo Aung, MD, MPH, FACP  Associate Professor, Department of Medicine, University of Texas Health Science Center at San Antonio; Adjunct Associate Professor of Public Health, University of Texas School of Public Health

KoKo Aung, MD, MPH, FACP is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS  Professor of Medicine (Endocrinology, Adj), Johns Hopkins School of Medicine; Affiliate Research Professor, Bioinformatics and Computational Biology Program, School of Computational Sciences, George Mason University; Principal, C/A Informatics, LLC

Arthur B Chausmer, MD, PhD, FACP, FACE, FACN, CNS is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Nutrition, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, Endocrine Society, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Mark Cooper, MBBS, PhD, FRACP  Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD  Professor of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

Additional Contributors

The editors wish to thank Magdi Salmon, MD, for his previous contributions to this article.

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