eMedicine Specialties > Endocrinology > Multiple Endocrine Disease and Miscellaneous Endocrine Disease

Polyglandular Autoimmune Syndrome, Type I: Follow-up

Author: Saleh A Aldasouqi, MD, FACP, FACE, Assistant Professor of Medicine, Associate Program Director, Department of Medicine, Division of Endocrinology, Michigan State University College of Human Medicine
Coauthor(s): Olakunle PA Akinsoto, MD, MB, BCh, Consulting Staff, Family Health Center, Jacksonville Medical Center; Serge A Jabbour, MD, Associate Professor, Department of Medicine, Division of Endocrinology, Thomas Jefferson University
Contributor Information and Disclosures

Updated: Jan 7, 2009

Follow-up

Further Inpatient Care

If evidence of hypothyroidism is present, perform an adrenal evaluation before any thyroid replacement. If replacement of thyroid hormones is urgent, draw blood for later adrenal evaluation, and administer steroids before starting thyroid replacement dosing.

Further Outpatient Care

Apart from the usual medications, enforce the following measures:
  • The patient's diet should be high in calcium, fresh fruits, and vegetables and low in simple carbohydrates.
  • In addition to any other stress management techniques, encourage moderate exercise. This is mainly relevant for patients with adrenal insufficiency.
  • Patients may need a dual-energy radiographic absorptiometry (DEXA) scan to assess any degree of osteoporosis due to long-term steroid use.
  • Inform patients about the symptoms of an acute exacerbation, such as dizziness, lightheadedness, abdominal pain, and nausea and vomiting.
  • In addition, make patients aware of the signs and symptoms of hypoparathyroidism, including muscle cramps or spasms.
  • If evidence of hypothyroidism exists, perform an adrenal evaluation before any thyroid replacement. If replacement of thyroid hormones is urgent or emergent, draw blood for later adrenal evaluation, and administer steroids before starting thyroid replacement dosing.

Inpatient & Outpatient Medications

These medications depend on the components present in individual patients and range from agents used for hormone replacement to medications employed to manage fungal infections and other complications/deficiencies.

Deterrence/Prevention

  • Strongly advise patients to wear medical alert bracelets indicating that they have adrenal insufficiency.
  • Provide patients with increased steroid coverage before surgeries or periods of stress (for example, in the case of a febrile illness).

Complications

  • Hypoparathyroidism
    • Cataracts
    • Laryngospasm
    • Basal ganglial calcification
    • Ventricular arrhythmias
    • Renal stones may arise from vitamin D use due to possible excessive urine Ca++ excretion. Urine calcium excretion may be monitored in these patients.
  • Addison disease
    • Arrhythmias secondary to electrolyte imbalance
    • Loss of libido
    • Psychotic illnesses
    • Hypoglycemic spells
    • Gastrointestinal complaints
    • Complications from treatment, such as osteoporosis or gastrointestinal ulceration with concurrent use of nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Other complications include the following:

Prognosis

The prognosis is variable, depending on how organs are affected and the severity of the disease.

Patient Education

  • Outpatient management should include patient education on the various components of polyglandular autoimmune (PGA) syndrome, type I, and the need to screen close relatives as appropriate. An important aspect of patient education is the provision of information about adrenal deficiency; subtle deficiency that goes unnoticed in normal, daily-life situations may become life-threatening in stressful situations.
  • See Further Outpatient Care.

Miscellaneous

Medicolegal Pitfalls

  • Failure to refer the patient to a specialist (usually an endocrinologist)
  • Failure to provide proper follow-up care
    • Polyglandular autoimmune (PGA) syndrome, type I, can have multiple pathologies.
    • It has an unpredictable outcome, because each pathology can occur anytime during the course of the disease.
    • Failure to see these patients as frequently as possible can result in missing the onset of a potentially fatal pathology.
  • Failure to provide appropriate genetic counseling

Special Concerns

When evidence of a second autoimmunity is present, consider that the patient may have polyglandular autoimmune (PGA) syndrome, type I, or PGA-II, because of the different prognoses in these syndromes.

 
Acknowledgments

I would like to thank Jinie Shirey at the Department of Medicine, College of Human Medicine, Michigan State University, East Lansing for manuscript assistance and preparation, and Laura Smith at the Medical Library, Sparrow Hospital, Lansing, Michigan, for assistance in reference retrieval.



More on Polyglandular Autoimmune Syndrome, Type I

Overview: Polyglandular Autoimmune Syndrome, Type I
Differential Diagnoses & Workup: Polyglandular Autoimmune Syndrome, Type I
Treatment & Medication: Polyglandular Autoimmune Syndrome, Type I
Follow-up: Polyglandular Autoimmune Syndrome, Type I
References
Further Reading
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References

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Keywords

polyglandular autoimmune syndrome, autoimmune, adrenal, autoimmune diseases, adrenal glands, Addison's disease, autoimmune disorders, adrenal gland, autoimmune disease, autoimmune disorder, candidiasis, Addison disease, auto immune, polyglandular autoimmune syndrome type I, polyglandular autoimmune syndrome type 1, autoimmune polyendocrine syndromes, APS, autoimmune polyendocrinopathy, autoimmune polyglandular syndrome, candidiasis ectodermal dysplasia, immunoendocrinopathy syndromes, PGA syndromes, polyglandular failure syndromes, endocrine gland insufficiency, autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy, APECED, Whitaker syndrome, PGA syndrome type I, PGA-I

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Contributor Information and Disclosures

Author

Saleh A Aldasouqi, MD, FACP, FACE, Assistant Professor of Medicine, Associate Program Director, Department of Medicine, Division of Endocrinology, Michigan State University College of Human Medicine
Saleh A Aldasouqi, MD, FACP, FACE is a member of the following medical societies: American Association of Clinical Endocrinologists and American College of Physicians
Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Takeda Honoraria Speaking and teaching; Amylin Grant/research funds Clinical Trial

Coauthor(s)

Olakunle PA Akinsoto, MD, MB, BCh, Consulting Staff, Family Health Center, Jacksonville Medical Center
Olakunle PA Akinsoto, MD, MB, BCh is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Medical Association
Disclosure: Nothing to disclose.

Serge A Jabbour, MD, Associate Professor, Department of Medicine, Division of Endocrinology, Thomas Jefferson University
Serge A Jabbour, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Medical Association, American Thyroid Association, Endocrine Society, and Pennsylvania Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Ghassem Pourmotabbed, MD†, Former Associate Professor, Department of Internal Medicine, Division of Endocrinology and Metabolism, University of Tennessee School of Medicine and Health Science Center
Ghassem Pourmotabbed, MD† is a member of the following medical societies: American Diabetes Association, American Federation for Medical Research, and Endocrine Society
Disclosure: Nothing to disclose.

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Disclosure: eMedicine Salary Employment

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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 Physician Executives, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, American Society for Bone and Mineral Research, American Society of Law Medicine and Ethics, Endocrine Society, and International Society for Clinical Densitometry
Disclosure: Nothing to disclose.

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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.

 
 
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