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Type II Polyglandular Autoimmune Syndrome Medication

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

Medication Summary

With the exception of antithyroid drugs for Graves disease, most medications listed here are essentially for replacement therapy.

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Glucocorticoids

Class Summary

Glucocorticoids are used in the replacement therapy associated with adrenal failure. Significant trauma can acutely increase the need for such treatment.

Hydrocortisone (Hydrocortone, Hydrocort, Cortef, Hydro-Tex)

 

Useful in treatment of diverse group of diseases, especially autoimmune and inflammatory diseases. Used for primary adrenal failure. Has weak mineralocorticoid activity. Individualize dosing.

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Thyroid hormones

Class Summary

These agents are used for thyroid replacement in hypothyroidism.

Levothyroxine (Synthroid, Levoxyl, Levothroid, Unithroid, Tirosint)

 

DOC due to stability, cost, lack of foreign-protein allergens, and long half-life (qd dosing). T4 converted to T3 intracellularly, and T4 administration produces both hormones. In active form, influences growth and maturation of tissues. Involved in normal growth, metabolism, and development.

Infants and children require more T4/kg than do adults.

Dosing depends on age and comorbidity.

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Antithyroid agents

Class Summary

These drugs act by inhibiting TPO-catalyzed reactions to block iodine organification and by inhibiting peripheral deiodination of T4/T3. (The last effect is seen only by propylthiouracil [PTU].)

Propylthiouracil (PTU)

 

Derivative of thiourea that inhibits organification of iodine by thyroid gland. Blocks oxidation of iodine in thyroid gland, thereby inhibiting thyroid hormone synthesis; inhibits T4 to T3 conversion (advantage over other agents). Ten times less active than methimazole.

Relatively safe in pregnancy and breastfeeding due to tight bond to plasma proteins.

Methimazole (Tapazole)

 

Inhibits thyroid hormone by blocking oxidation of iodine in thyroid gland. However, not known to inhibit peripheral conversion of thyroid hormone. Taper gradually to the minimum dose required to keep the patient clinically euthyroid and to avoid fetal hypothyroidism. Cases of fetal aplasia cutis are reported.

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Antidiabetic agents

Class Summary

These agents are used for type 1 diabetes mellitus replacement.

Insulin regular human (Humulin, Novolin), NPH Insulin (Humulin, Novolin), Long-acting basal insulin analogs (detemir, glargine), Fast-acting prandial insulin analogs (lispro, aspart, glulisine).

 

Stimulates proper utilization of glucose by the cells and reduces blood sugar levels. Wide variety derived from pork, beef, and synthetic human derivatives. Various preparations with variable onsets of actions; shortest and quickest is lispro insulin, and longest acting is ultralente insulin. Not administered PO because becomes denatured by acid and intestinal peptidases. Can be administered IV/IM/SC. Nasal administration may be available soon, depending on required preparation.

Dosing individualized based on lifestyle, dietary compliance, infections, and surgeries.

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Mineralocorticoids

Class Summary

These are employed in partial replacement therapy for primary and secondary adrenocortical insufficiency.

Fludrocortisone (Florinef)

 

Mineralocorticoid required for conservation of Na and renal loss of K. Maintains blood pressure and intravascular/extracellular volume.

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Vitamins

Class Summary

Vitamin B-12 replacement in pernicious anemia. Megaloblastic anemia must be further evaluated to differentiate folate deficiency from vitamin B-12 deficiency, because the latter requires life-long treatment. When cyanocobalamin is deficient mainly due to malabsorption, it must be replaced via the NG route. Hydroxocobalamin is the more potent vitamin B-12 variant, because it forms a tight bond with plasma proteins and stays in circulation longer. Hydroxocobalamin may be a good complexing agent for cyanide poisoning. Possible effective antidote.

Cyanocobalamin (Crystamine, Cyomin, Crysti 1000, Nascobal)

 

Deoxyadenosylcobalamin and hydroxocobalamin are active forms of vitamin B-12 in humans. Vitamin B-12 is synthesized by microbes but not by humans or plants. Vitamin B-12 deficiency may result from intrinsic factor deficiency (pernicious anemia), partial or total gastrectomy, or diseases of the distal ileum.

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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.

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