Addison Disease Questions & Answers

1. Munir S, Quintanilla Rodriguez BS, Waseem M. Addison Disease. StatPearls. 2022 Jan. [QxMD MEDLINE Link]. [Full Text].

2. Addison T. On the Constitutional and Local Effects of Disease of the Supra-renal Capsules. London, UK: Samuel Highley; 1855.

3. Oelkers W, Diederich S, Bahr V. Diagnosis and therapy surveillance in Addison's disease: rapid adrenocorticotropin (ACTH) test and measurement of plasma ACTH, renin activity, and aldosterone. J Clin Endocrinol Metab. 1992 Jul. 75(1):259-64. [QxMD MEDLINE Link]. [Full Text].

4. Debono M, Ross RJ. Doses and steroids to be used in primary and central hypoadrenalism. Ann Endocrinol (Paris). 2007 Sep. 68(4):265-7. [QxMD MEDLINE Link].

5. Reisch N, Arlt W. Fine tuning for quality of life: 21st century approach to treatment of Addison's disease. Endocrinol Metab Clin North Am. 2009 Jun. 38(2):407-18, ix-x. [QxMD MEDLINE Link].

6. Olafsson AS, Sigurjonsdottir HA. INCREASING PREVALENCE OF ADDISON DISEASE: RESULTS FROM A NATIONWIDE STUDY. Endocr Pract. 2016 Jan. 22 (1):30-5. [QxMD MEDLINE Link].

7. Hong AR, Ryu OH, Kim SY, Kim SW, Korean Adrenal Gland and Endocrine Hypertension Study Group, Korean Endocrine Society. Characteristics of Korean Patients with Primary Adrenal Insufficiency: A Registry-Based Nationwide Survey in Korea. Endocrinol Metab (Seoul). 2017 Dec. 32 (4):466-74. [QxMD MEDLINE Link]. [Full Text].

8. Kyriazopoulou V. Glucocorticoid replacement therapy in patients with Addison's disease. Expert Opin Pharmacother. 2007 Apr. 8(6):725-9. [QxMD MEDLINE Link].

9. Bergthorsdottir R, Leonsson-Zachrisson M, Oden A, et al. Premature mortality in patients with Addison's disease: a population-based study. J Clin Endocrinol Metab. 2006 Dec. 91(12):4849-53. [QxMD MEDLINE Link]. [Full Text].

10. Skov J, Sundstrom A, Ludvigsson JF, Kampe O, Bensing S. Sex-Specific Risk of Cardiovascular Disease in Autoimmune Addison Disease-A Population-Based Cohort Study. J Clin Endocrinol Metab. 2019 Jun 1. 104 (6):2031-40. [QxMD MEDLINE Link]. [Full Text].

11. Hemminki K, Li X, Sundquist J, et al. Subsequent autoimmune or related disease in asthma patients: clustering of diseases or medical care?. Ann Epidemiol. 2010 Mar. 20(3):217-22. [QxMD MEDLINE Link].

12. White K, Arlt W. Adrenal crisis in treated Addison's disease: a predictable but under-managed event. Eur J Endocrinol. 2010 Jan. 162(1):115-20. [QxMD MEDLINE Link].

13. Chantzichristos D, Persson A, Eliasson B, et al. Mortality in patients with diabetes mellitus and Addison's disease: a nationwide, matched, observational cohort study. Eur J Endocrinol. 2017 Jan. 176 (1):31-9. [QxMD MEDLINE Link].

14. Vogt EC, Breivik L, Royrvik EC, Grytaas M, Husebye ES, Oksnes M. Primary Ovarian Insufficiency in Women With Addison's Disease. J Clin Endocrinol Metab. 2021 Jun 16. 106 (7):e2656-63. [QxMD MEDLINE Link]. [Full Text].

15. Barnett AH, Espiner EA, Donald RA. Patients presenting with Addison's disease need not be pigmented. Postgrad Med J. 1982 Nov. 58(685):690-2. [QxMD MEDLINE Link].

16. McBrien DJ. Steatorrhea in Addison's disease. Lancet. 1963. Vol I:25-6.

17. Van Dellen RG, Purnell DC. Hyperkalemic paralysis in Addison's disease. Mayo Clin Proc. 1969 Dec. 44(12):904-14.

18. Likhari T, Magzoub S, Griffiths MJ, et al. Screening for Addison's disease in patients with type 1 diabetes mellitus and recurrent hypoglycaemia. Postgrad Med J. 2007 Jun. 83(980):420-1. [QxMD MEDLINE Link].

19. Guo YK, Yang ZG, Li Y, et al. Addison's disease due to adrenal tuberculosis: contrast-enhanced CT features and clinical duration correlation. Eur J Radiol. 2007 Apr. 62(1):126-31. [QxMD MEDLINE Link].

20. Husebye E, Lovas K. Pathogenesis of primary adrenal insufficiency. Best Pract Res Clin Endocrinol Metab. 2009 Apr. 23(2):147-57. [QxMD MEDLINE Link].

21. Husebye ES, Bratland E, Bredholt G, et al. The substrate-binding domain of 21-hydroxylase, the main autoantigen in autoimmune Addison's disease, is an immunodominant T cell epitope. Endocrinology. 2006 May. 147(5):2411-6. [QxMD MEDLINE Link]. [Full Text].

22. Wolff AS, Myhr KM, Vedeler CA, et al. Fc-gamma receptor polymorphisms are not associated with autoimmune Addison's disease. Scand J Immunol. 2007 Jun. 65(6):555-8. [QxMD MEDLINE Link].

23. Roycroft M, Fichna M, McDonald D, et al. The tryptophan 620 allele of the lymphoid tyrosine phosphatase (PTPN22 gene) predisposes to autoimmune Addison's disease. Clin Endocrinol (Oxf). 2008 Aug 15. [QxMD MEDLINE Link].

24. Elfstrom P, Montgomery SM, Kampe O, et al. Risk of primary adrenal insufficiency in patients with celiac disease. J Clin Endocrinol Metab. 2007 Sep. 92(9):3595-8. [QxMD MEDLINE Link]. [Full Text].

25. Betterle C, Lazzarotto F, Spadaccino AC, et al. Celiac disease in North Italian patients with autoimmune Addison's disease. Eur J Endocrinol. 2006 Feb. 154(2):275-9. [QxMD MEDLINE Link].

26. Biagi F, Campanella J, Soriani A, et al. Prevalence of coeliac disease in Italian patients affected by Addison's disease. Scand J Gastroenterol. 2006 Mar. 41(3):302-5. [QxMD MEDLINE Link].

27. Wolff AS, Erichsen MM, Meager A, et al. Autoimmune polyendocrine syndrome type 1 in Norway: phenotypic variation, autoantibodies, and novel mutations in the autoimmune regulator gene. J Clin Endocrinol Metab. 2007 Feb. 92(2):595-603. [QxMD MEDLINE Link].

28. Magitta NF, Boe Wolff AS, Johansson S, et al. A coding polymorphism in NALP1 confers risk for autoimmune Addison's disease and type 1 diabetes. Genes Immun. 2008 Oct 23. [QxMD MEDLINE Link].

29. Gombos Z, Hermann R, Kiviniemi M, et al. Analysis of extended human leukocyte antigen haplotype association with Addison's disease in three populations. Eur J Endocrinol. 2007 Dec. 157(6):757-61. [QxMD MEDLINE Link].

30. Glasgow BJ, Steinsapir KD, Anders K, et al. Adrenal pathology in the acquired immune deficiency syndrome. Am J Clin Pathol. 1985 Nov. 84(5):594-7. [QxMD MEDLINE Link].

31. Greene LW, Cole W, Greene JB, et al. Adrenal insufficiency as a complication of the acquired immunodeficiency syndrome. Ann Intern Med. 1984 Oct. 101(4):497-8. [QxMD MEDLINE Link].

32. Membreno L, Irony I, Dere W, et al. Adrenocortical function in acquired immunodeficiency syndrome. J Clin Endocrinol Metab. 1987 Sep. 65(3):482-7. [QxMD MEDLINE Link].

33. Mukhopadhya A, Danda S, Huebner A, et al. Mutations of the AAAS gene in an Indian family with Allgrove's syndrome. World J Gastroenterol. 2006 Aug 7. 12(29):4764-6. [QxMD MEDLINE Link].

34. Migeon CJ, Kenny EM, Kowarski A, et al. The syndrome of congenital adrenocortical unresponsiveness to ACTH. Report of six cases. Pediatr Res. 1968 Nov. 2(6):501-13. [QxMD MEDLINE Link].

35. Babu K, Murthy KR, Babu N, et al. Triple A syndrome with ophthalmic manifestations in two siblings. Indian J Ophthalmol. 2007 Jul-Aug. 55(4):304-6. [QxMD MEDLINE Link]. [Full Text].

36. Mukherjee S, Newby E, Harvey JN. Adrenomyeloneuropathy in patients with 'Addison's disease': genetic case analysis. J R Soc Med. 2006 May. 99(5):245-9. [QxMD MEDLINE Link].

37. Dias RP, Chan LF, Metherell LA, et al. Isolated Addison's disease is unlikely to be caused by mutations in MC2R, MRAP or STAR, three genes responsible for familial glucocorticoid deficiency. Eur J Endocrinol. 2010 Feb. 162(2):357-9. [QxMD MEDLINE Link]. [Full Text].

38. Baker BY, Lin L, Kim CJ, et al. Nonclassic congenital lipoid adrenal hyperplasia: a new disorder of the steroidogenic acute regulatory protein with very late presentation and normal male genitalia. J Clin Endocrinol Metab. 2006 Dec. 91(12):4781-5. [QxMD MEDLINE Link]. [Full Text].

39. Dackis CA, Gurpegui M, Pottash AL, et al. Methadone induced hypoadrenalism. Lancet. 1982 Nov 20. 2(8308):1167.

40. Sekiguchi Y, Hara Y, Matsuoka H, et al. Sibling cases of Addison's disease caused by DAX-1 gene mutations. Intern Med. 2007. 46(1):35-9. [QxMD MEDLINE Link]. [Full Text].

41. Elansary EH, Earis JE. Rifampicin and adrenal crisis. Br Med J (Clin Res Ed). 1983 Jun 11. 286(6381):1861-2.

42. Kyriazopoulou V, Parparousi O, Vagenakis AG. Rifampicin-induced adrenal crisis in addisonian patients receiving corticosteroid replacement therapy. J Clin Endocrinol Metab. 1984 Dec. 59(6):1204-6.

43. Demers LM, Whitley RJ. Function of the adrenal cortex: protocol for the rapid ACTH test. In: Burtis CA, Ashwood ER, eds. Tietz Textbook of Clinical Chemistry. 3rd ed. Philadelphia, Pa: WB Saunders; 1999. Vol 43: 1530-60.

44. Dickstein G, Shechner C, Nicholson WE, et al. Adrenocorticotropin stimulation test: effects of basal cortisol level, time of day, and suggested new sensitive low dose test. J Clin Endocrinol Metab. 1991 Apr. 72(4):773-8. [QxMD MEDLINE Link].

45. Dluhy RG, Himathongkam T, Greenfield M. Rapid ACTH test with plasma aldosterone levels. Improved diagnostic discrimination. Ann Intern Med. 1974 Jun. 80(6):693-6.

46. Lindholm J, Kehlet H. Re-evaluation of the clinical value of the 30 min ACTH test in assessing the hypothalamic-pituitary-adrenocortical function. Clin Endocrinol (Oxf). 1987 Jan. 26(1):53-9. [QxMD MEDLINE Link].

47. May ME, Carey RM. Rapid adrenocorticotropic hormone test in practice. Retrospective review. Am J Med. 1985 Dec. 79(6):679-84. [QxMD MEDLINE Link].

48. Barnett AH, Donald RA, Espiner EA. High concentrations of thyroid-stimulating hormone in untreated glucocorticoid deficiency: indication of primary hypothyroidism?. Br Med J (Clin Res Ed). 1982 Jul 17. 285(6336):172-3. [QxMD MEDLINE Link].

49. Gharib H, Hodgson SF, Gastineau CF, et al. Reversible hypothyroidism in Addison's disease. Lancet. 1972 Oct 7. 2(7780):734-6. [QxMD MEDLINE Link].

50. Ma ES, Yang ZG, Li Y, et al. Tuberculous Addison's disease: morphological and quantitative evaluation with multidetector-row CT. Eur J Radiol. 2007 Jun. 62(3):352-8. [QxMD MEDLINE Link].

51. Lovas K, Husebye ES. Continuous subcutaneous hydrocortisone infusion in Addison's disease. Eur J Endocrinol. 2007 Jul. 157(1):109-12. [QxMD MEDLINE Link].

52. Reznik Y, Barat P, Bertherat J, et al. SFE/SFEDP adrenal insufficiency French consensus: Introduction and handbook. Ann Endocrinol (Paris). 2018 Jan 12. [QxMD MEDLINE Link].

53. Lovas K, Gjesdal CG, Christensen M, et al. Glucocorticoid replacement therapy and pharmacogenetics in Addison's disease: effects on bone. Eur J Endocrinol. 2009 Jun. 160(6):993-1002. [QxMD MEDLINE Link].

54. Schulz J, Frey KR, Cooper MS, et al. Reduction in daily hydrocortisone dose improves bone health in primary adrenal insufficiency. Eur J Endocrinol. 2016 Apr. 174 (4):531-8. [QxMD MEDLINE Link].

55. Chandy DD, Bhatia E. BONE MINERAL DENSITY IN PATIENTS WITH ADDISON DISEASE ON REPLACEMENT THERAPY WITH PREDNISOLONE. Endocr Pract. 2016 Apr. 22 (4):434-9. [QxMD MEDLINE Link].

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

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

Disclosure: Nothing to disclose.

Steven B Nagelberg, MD Clinical Professor, Department of Medicine, Division of Endocrinology and Metabolism, Drexel University College of Medicine

Steven B Nagelberg, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, American Medical Association, Endocrine Society, Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Sylvester Odeke, MD, FACE Endocrinologist, Banner Health Physicians Colorado, LLC, North Colorado Medical Center

Sylvester Odeke, MD, FACE is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, North Carolina Medical Society

Disclosure: Nothing to disclose.

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.

Romesh Khardori, MD, PhD, FACP (Retired) Professor, Division of Endocrinology, Diabetes and Metabolism, 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.

This chapter is dedicated to the late Dr. James C. Melby.

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