Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Type II Polyglandular Autoimmune Syndrome Follow-up

  • Author: Surendra Sivarajah, MD; Chief Editor: Romesh Khardori, MD, PhD, FACP  more...
 
Updated: Aug 28, 2014
 

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.

Next

Inpatient & Outpatient Medications

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

Previous
Next

Complications

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

Previous
Next

Prognosis

See Further Inpatient Care.

Previous
Next

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.

Previous
 
Contributor Information and Disclosures
Author

Surendra Sivarajah, MD Interim Chief, Section of Endocrinology and Metabolism, The Reading Hospital and Medical Center

Surendra Sivarajah, MD is a member of the following medical societies: American College of Physicians, American Medical Association, Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

Olakunle P A Akinsoto, MD, MB, BCh Consulting Staff, Family Health Center

Olakunle P A Akinsoto, MD, MB, BCh is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Medical Association

Disclosure: Nothing to disclose.

Chris Y Fan, MD Assistant Professor of Medicine, Division of Endocrinology, Diabetes, and Metabolism, Pennsylvania State University College of Medicine, Practice Site Director, Endocrinology and Nephrology Clinic, Hershey Medical Center

Chris Y Fan, MD is a member of the following medical societies: American College of Physicians, American Diabetes Association, American Medical Association, Endocrine Society

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: Received salary from Medscape for employment. for: Medscape.

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 Nutrition, American Society for Bone and Mineral Research, International Society for Clinical Densitometry, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Informatics Association, Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

Romesh Khardori, MD, PhD, FACP Professor of Endocrinology, Director of Training Program, Division of Endocrinology, Diabetes and Metabolism, Strelitz Diabetes and Endocrine Disorders Institute, Department of Internal Medicine, Eastern Virginia Medical School

Romesh Khardori, MD, PhD, FACP is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, Endocrine Society

Disclosure: Nothing to disclose.

Additional Contributors

Ghassem Pourmotabbed, MD, MD 

Ghassem Pourmotabbed, MD, MD is a member of the following medical societies: American Diabetes Association, American Federation for Medical Research, Endocrine Society

Disclosure: Nothing to disclose.

References
  1. Obermayer-Straub P, Manns MP. Autoimmune polyglandular syndromes. Baillieres Clin Gastroenterol. 1998 Jun. 12(2):293-315. [Medline].

  2. Eisenbarth GS, Gottlieb PA. Autoimmune polyendocrine syndromes. N Engl J Med. 2004 May 13. 350(20):2068-79. [Medline].

  3. Baker JR Jr. Autoimmune endocrine disease. JAMA. 1997 Dec 10. 278(22):1931-7. [Medline].

  4. Betterle C, Lazzarotto F, Presotto F. Autoimmune polyglandular syndrome Type 2: the tip of an iceberg?. Clin Exp Immunol. 2004 Aug. 137(2):225-33. [Medline]. [Full Text].

  5. Ramos-Lopez E, Lange B, Kahles H, et al. Insulin gene polymorphisms in type 1 diabetes, Addison's disease and the polyglandular autoimmune syndrome type II. BMC Med Genet. 2008 Jul 11. 9:65. [Medline]. [Full Text].

  6. Cooper GS, Stroehla BC. The epidemiology of autoimmune diseases. Autoimmun Rev. 2003 May. 2(3):119-25. [Medline].

  7. Borgaonkar MR, Morgan DG. Primary biliary cirrhosis and type II autoimmune polyglandular syndrome. Can J Gastroenterol. 1999 Nov. 13(9):767-70. [Medline].

  8. de Graaff LC, Smit JW, Radder JK. Prevalence and clinical significance of organ-specific autoantibodies in type 1 diabetes mellitus. Neth J Med. 2007 Jul-Aug. 65(7):235-47. [Medline].

  9. Förster G, Krummenauer F, Kühn I, et al. [Polyglandular autoimmune syndrome type II: epidemiology and forms of manifestation]. Dtsch Med Wochenschr. 1999 Dec 10. 124(49):1476-81. [Medline].

  10. Bizzaro N. The predictive significance of autoantibodies in organ-specific autoimmune diseases. Clin Rev Allergy Immunol. 2008 Jun. 34(3):326-31. [Medline].

  11. Rakel RE, ed. Conn's Current Therapy. Philadelphia, Pa: Saunders; 1999. 630-54.

  12. Cooper DS, Rivkees SA. Putting propylthiouracil in perspective. J Clin Endocrinol Metab. 2009 Jun. 94(6):1881-2. [Medline].

  13. Eisenbarth G, Verge C. Immunoendocrinopathy syndromes. Wilson JD, ed. Williams Textbook of Endocrinology. 9th ed. Philadelphia, Pa: Saunders; 1998. 1651-9.

  14. Lachmann PJ, Rosen F. Autoimmune endocrine disease. Lachmann PJ, ed. Clinical Aspects of Immunology. 5th ed. Boston, Mass: Blackwell Scientific Pubs; 1993. 2001-2.

  15. Muir A, Katz D, McClaren N. Polyglandular failure syndromes. DeGroot LJ, ed. DeGroot's Endrocrinology. 3rd ed. Philadelphia, Pa: Saunders; 1995. 3013-22.

  16. US Food and Drug Administration. Information for Healthcare Professionals - Propylthiouracil-Induced Liver Failure. US Department of Health and Human Services. Available at http://www.fda.gov/Drugs/DrugSafety/PostmarketDrugSafetyInformationforPatientsandProviders/DrugSafetyInformationforHeathcareProfessionals/ucm162701.htm. Accessed: April 9, 2013.

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.