Клинические рекомендации Российской ассоциации эндокринологов по диагностике и лечению первичной надпочечниковой недостаточности у взрослых пациентов (проект)

1. Hahner S, Allolio B. Therapeutic management of adrenal insufficiency. Best Pract Res Clin Endocrinol Metab 2009; 23: 167–79.
2. Meyer G, Hackemann A, Penna-Martinez M, Badenhoop K. What affects the quality of life in autoimmune Addison’s disease? Horm Metab Res 2013; 45: 92–5.
3. Chakera AJ, Vaidya B. Addison disease in adults: diagnosis and management. Am J Med 2010; 123: 409–13.
4. Meyer G, Neumann K, Badenhoop K, Linder R. Increasing prevalence of Addison’s disease in German females: health insurance data 2008–2012. Eur J Endocrinol 2014; 170: 367–73.
5. Bornstein SR. Predisposing factors for adrenal insufficiency. N Engl J Med 2009; 360: 2328–39.
6. Мельниченко Г.А. и др. Клинические рекомендации Российской ассоциации эндокринологов по диагностике и лечебно-профилактическим мероприятиям при врожденной дисфункции коры надпочечников у пациентов во взрослом возрасте. Consi-lium Medicum. 2016; 18 (4): 8–19. / Melnichenko G.A. et al. Russian Association of Endocrinologists clinical practice guidelines for diagnosis, treatment and preventive measures in congenital adrenal hyperplasia due to 21-hydroxylase deficiency patients in adulthood. Consilium Medicum. 2016; 18 (4): 8–19. [in Russian]
7. Kazlauskaite R, Evans AT, Villabona CV et al. Corticotropin tests for hypothalamic-pituitary-adrenal insufficiency: a metaanalysis. J Clin Endocrinol Metab 2008; 93: 4245–53.
8. Lee MK, Vasikaran S, Doery JC et al. Cortisol: ACTH ratio to test for primary hypoadrenalism: a pilot study. Postgrad Med J 2013; 89: 617–20.
9. Cho HY, Kim JH, Kim SW et al. Different cut-off values of the insulin tolerance test, the high-dose short Synacthen test (250 mg) and the low-dose short Synacthen test (1 mg) in assessing central adrenal insufficiency. Clin Endocrinol (Oxf) 2014; 81: 77–84.
10. Bornstein SR, Allolio B, Arlt W et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101 (2): 364–89.
11. Gundgurthi A, Garg MK, Dutta MK, Pakhetra R. Intramuscular ACTH stimulation test for assessment of adrenal function. J Assoc Physicians India 2013; 61: 320–4.
12. Gagliardi L, Ho JT, Torpy DJ. Corticosteroid-binding globulin: the clinical significance of altered levels and heritable mutations. Mol Cell Endocrinol 2010; 316: 24–34.
13. Lebbe M, Arlt W. What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy? Clin Endocrinol (Oxf) 2013; 78: 497–502.
14. Speiser PW, Azziz R, Baskin LS et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 2010; 95: 4133–60.
15. El-Farhan N, Pickett A, Ducroq D et al. Method-specific serum cortisol responses to the adrenocorticotrophin test: comparison of gas chromatography-mass spectrometry and five automated immunoassays. Clin Endocrinol (Oxf) 2013; 78: 673–80.
16. Дедов И.И., Мельниченко Г.А., Фадеев В.В. Эндокринология. 3-е изд., перераб. и доп. М.: Литтерра, 2015. / Dedov I.I., Mel'nichenko G.A., Fadeev V.V. Endokrinologiia. 3-e izd., pererab. i dop. M.: Litterra, 2015. [in Russian]
17. Baker PR, Baschal EE, Fain PR et al. Haplotype analysis discriminates genetic risk for DR3-associated endocrine autoimmunity and helps define extreme risk for Addison’s disease. J Clin Endocrinol Metab 2010; 95: E263–E270.
18. Coco G, Dal Pra C, Presotto F et al. Estimated risk for developing autoimmune Addison’s disease in patients with adrenal cortex autoantibodies. J Clin Endocrinol Metab 2006; 91: 1637–45.
19. Meager A, Visvalingam K, Peterson P et al. Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med 2006; 3: e289.
20. Horn MA, Erichsen MM, Wolff AS et al. Screening for X-linked adrenoleukodystrophy among adult men with Addison’s disease. Clin Endocrinol (Oxf) 2013; 79: 316–20.
21. Ekman B, Bachrach-Lindström M, Lindström T et al. A randomized, double-blind, crossover study comparing two- and four-dose hydrocortisone regimen with regard to quality of life, cortisol and ACTH profiles in patients with primary adrenal insufficiency. Clin Endocrinol (Oxf) 2012; 77: 18–25.
22. Forss M, Batcheller G, Skrtic S, Johannsson G. Current practice of glucocorticoid replacement therapy and patient-perceived health outcomes in adrenal insufficiency – a worldwide patient survey. BMC Endocr Disord 2012; 12: 8.
23. Johannsson G, Nilsson AG, Bergthorsdottir R et al. Improved cortisol exposure-time profile and outcome in patients with adrenal insufficiency: a prospective randomized trial of a novel hydrocortisone dual-release formulation. J Clin Endocrinol Metab 2012; 97: 473–81.
24. Miller WL, Auchus RJ. The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders. Endocr Rev 2011; 32: 81–151.
25. Methlie P, Husebye EE, Hustad S et al. Grapefruit juice and licorice increase cortisol availability in patients with Addison’s disease. Eur J Endocrinol 2011; 165: 761–9.
26. Ross IL, Bergthorsdottir R, Levitt N et al. Cardiovascular risk factors in patients with Addison’s disease: a comparative study of South African and Swedish patients. PLoS One 2014; 9: e90768.
27. Inder WJ, Meyer C, Hunt PJ. Management of hypertension and heart failure in patients with Addison’s disease. Clin Endocrinol (Oxf) 2015; 82: 789–92.
28. Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol 2015; 3: 216–26.
29. Allolio B. Extensive expertise in endocrinology: adrenal crisis. Eur J Endocrinol 2015; 172: R115–R124.
30. Hahner S, Spinnler C, Fassnacht M et al. High incidence of adrenal crisis in educated patients with chronic adrenal insufficiency: a prospective study. J Clin Endocrinol Metab 2015; 100: 407–16.
31. Reisch N, Willige M, Kohn D et al. Frequency and causes of adrenal crises over lifetime in patients with 21-hydroxylase deficiency. Eur J Endocrinol 2012; 167: 35–42.
32. Chortis V, Taylor AE, Schneider P et al. Mitotane therapy in adrenocortical cancer induces CYP3A4 and inhibits 5a-reductase, explaining the need for personalized glucocorticoid and androgen replacement. J Clin Endocrinol Metab 2013; 98: 161–71.
33. Quinkler M, Hahner S. What is the best long-term management strategy for patients with primary adrenal insufficiency? Clin Endocrinol (Oxf) 2012; 76: 21–5.
34. Boonen E, Vervenne H, Meersseman P et al. Reduced cortisol metabolism during critical illness. N Engl J Med 2013; 368: 1477–88.
35. Hahner S, Burger-Stritt S, Allolio B. Subcutaneous hydrocortisone administration for emergency use in adrenal insufficiency. Eur J Endocrinol 2013; 169: 147–54.
36. Hahner S, Hemmelmann N, Quinkler M et al. Timelines in the management of adrenal crisis – targets, limits and reality. Clin Endocrinol (Oxf) 2015; 82: 497–502.
37. Repping-Wuts HJ, Stikkelbroeck NM, Noordzij A et al. A glucocorticoid education group meeting: an effective strategy for improving self-management to prevent adrenal crisis. Eur J Endocrinol 2013; 169: 17–22.
38. Quinkler M, Dahlqvist P, Husebye ES, Kämpe O. A European Emergency Card for adrenal insufficiency can save lives. Eur J Intern Med 2015; 26: 75–6.
39. Reato G, Morlin L, Chen S et al. Premature ovarian failure in patients with autoimmune Addison’s disease: clinical, genetic, and immunological evaluation. J Clin Endocrinol Metab 2011; 96: E1255–E1261.
40. Falorni A, Brozzetti A, Aglietti MC et al. Progressive decline of residual follicle pool after clinical diagnosis of autoimmune ovarian insufficiency. Clin Endocrinol (Oxf) 2012; 77: 453–8.
41. Charmandari E, Nicolaides NC, Chrousos GP. Adrenal insufficiency. Lancet 2014; 383: 2152–67.
42. Christiansen JJ, Bruun JM, Christiansen JS et al. Long-term DHEA substitution in female adrenocortical failure, body composition, muscle function, and bone metabolism: a randomized trial. Eur J Endocrinol 2011; 165: 293–300.
43. Keevil BG. Novel liquid chromatography tandem mass spectrometry (LC-MS/MS) methods for measuring steroids. Best Pract Res Clin Endocrinol Metab 2013; 27: 663–74.
44. Mallappa A, Sinaii N, Kumar P et al. A phase 2 study of Chronocort, a modified-release formulation of hydrocortisone. J Clin Endocrinol Metab 2015; 100: 1137–45.
45. Oksnes M, Björnsdottir S, Isaksson M et al. Continuous subcutaneous hydrocortisone infusion versus oral hydrocortisone replacement for treatment of Addison’s disease: a randomized clinical trial. J Clin Endocrinol Metab 2014; 99: 1665–74.
46. Gagliardi L, Nenke MA, Thynne TR et al. Continuous subcutaneous hydrocortisone infusion therapy in Addison’s disease: a randomized, placebo-controlled clinical trial. J Clin Endocrinol Metab 2014; 99: 4149–57.
47. Russell GM, Durant C, Ataya A et al. Subcutaneous pulsatile glucocorticoid replacement therapy. Clin Endocrinol (Oxf) 2014; 81: 289–93.
48. Gan EH, McArthur K, Mitchell AL et al. Residual adrenal function in autoimmune Addison’s disease: improvement after tetracosactide (ACTH1-24) treatment. J Clin Endocrinol Metab 2014; 99: 111–8.
49. Husebye ES et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014; 275: 104–15.

________________________________________________

1. Hahner S, Allolio B. Therapeutic management of adrenal insufficiency. Best Pract Res Clin Endocrinol Metab 2009; 23: 167–79.
2. Meyer G, Hackemann A, Penna-Martinez M, Badenhoop K. What affects the quality of life in autoimmune Addison’s disease? Horm Metab Res 2013; 45: 92–5.
3. Chakera AJ, Vaidya B. Addison disease in adults: diagnosis and management. Am J Med 2010; 123: 409–13.
4. Meyer G, Neumann K, Badenhoop K, Linder R. Increasing prevalence of Addison’s disease in German females: health insurance data 2008–2012. Eur J Endocrinol 2014; 170: 367–73.
5. Bornstein SR. Predisposing factors for adrenal insufficiency. N Engl J Med 2009; 360: 2328–39.
6. Melnichenko G.A. et al. Russian Association of Endocrinologists clinical practice guidelines for diagnosis, treatment and preventive measures in congenital adrenal hyperplasia due to 21-hydroxylase deficiency patients in adulthood. Consilium Medicum. 2016; 18 (4): 8–19. [in Russian]
7. Kazlauskaite R, Evans AT, Villabona CV et al. Corticotropin tests for hypothalamic-pituitary-adrenal insufficiency: a metaanalysis. J Clin Endocrinol Metab 2008; 93: 4245–53.
8. Lee MK, Vasikaran S, Doery JC et al. Cortisol: ACTH ratio to test for primary hypoadrenalism: a pilot study. Postgrad Med J 2013; 89: 617–20.
9. Cho HY, Kim JH, Kim SW et al. Different cut-off values of the insulin tolerance test, the high-dose short Synacthen test (250 mg) and the low-dose short Synacthen test (1 mg) in assessing central adrenal insufficiency. Clin Endocrinol (Oxf) 2014; 81: 77–84.
10. Bornstein SR, Allolio B, Arlt W et al. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101 (2): 364–89.
11. Gundgurthi A, Garg MK, Dutta MK, Pakhetra R. Intramuscular ACTH stimulation test for assessment of adrenal function. J Assoc Physicians India 2013; 61: 320–4.
12. Gagliardi L, Ho JT, Torpy DJ. Corticosteroid-binding globulin: the clinical significance of altered levels and heritable mutations. Mol Cell Endocrinol 2010; 316: 24–34.
13. Lebbe M, Arlt W. What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy? Clin Endocrinol (Oxf) 2013; 78: 497–502.
14. Speiser PW, Azziz R, Baskin LS et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab 2010; 95: 4133–60.
15. El-Farhan N, Pickett A, Ducroq D et al. Method-specific serum cortisol responses to the adrenocorticotrophin test: comparison of gas chromatography-mass spectrometry and five automated immunoassays. Clin Endocrinol (Oxf) 2013; 78: 673–80.
16. Dedov I.I., Mel'nichenko G.A., Fadeev V.V. Endokrinologiia. 3-e izd., pererab. i dop. M.: Litterra, 2015. [in Russian]
17. Baker PR, Baschal EE, Fain PR et al. Haplotype analysis discriminates genetic risk for DR3-associated endocrine autoimmunity and helps define extreme risk for Addison’s disease. J Clin Endocrinol Metab 2010; 95: E263–E270.
18. Coco G, Dal Pra C, Presotto F et al. Estimated risk for developing autoimmune Addison’s disease in patients with adrenal cortex autoantibodies. J Clin Endocrinol Metab 2006; 91: 1637–45.
19. Meager A, Visvalingam K, Peterson P et al. Anti-interferon autoantibodies in autoimmune polyendocrinopathy syndrome type 1. PLoS Med 2006; 3: e289.
20. Horn MA, Erichsen MM, Wolff AS et al. Screening for X-linked adrenoleukodystrophy among adult men with Addison’s disease. Clin Endocrinol (Oxf) 2013; 79: 316–20.
21. Ekman B, Bachrach-Lindström M, Lindström T et al. A randomized, double-blind, crossover study comparing two- and four-dose hydrocortisone regimen with regard to quality of life, cortisol and ACTH profiles in patients with primary adrenal insufficiency. Clin Endocrinol (Oxf) 2012; 77: 18–25.
22. Forss M, Batcheller G, Skrtic S, Johannsson G. Current practice of glucocorticoid replacement therapy and patient-perceived health outcomes in adrenal insufficiency – a worldwide patient survey. BMC Endocr Disord 2012; 12: 8.
23. Johannsson G, Nilsson AG, Bergthorsdottir R et al. Improved cortisol exposure-time profile and outcome in patients with adrenal insufficiency: a prospective randomized trial of a novel hydrocortisone dual-release formulation. J Clin Endocrinol Metab 2012; 97: 473–81.
24. Miller WL, Auchus RJ. The molecular biology, biochemistry, and physiology of human steroidogenesis and its disorders. Endocr Rev 2011; 32: 81–151.
25. Methlie P, Husebye EE, Hustad S et al. Grapefruit juice and licorice increase cortisol availability in patients with Addison’s disease. Eur J Endocrinol 2011; 165: 761–9.
26. Ross IL, Bergthorsdottir R, Levitt N et al. Cardiovascular risk factors in patients with Addison’s disease: a comparative study of South African and Swedish patients. PLoS One 2014; 9: e90768.
27. Inder WJ, Meyer C, Hunt PJ. Management of hypertension and heart failure in patients with Addison’s disease. Clin Endocrinol (Oxf) 2015; 82: 789–92.
28. Bancos I, Hahner S, Tomlinson J, Arlt W. Diagnosis and management of adrenal insufficiency. Lancet Diabetes Endocrinol 2015; 3: 216–26.
29. Allolio B. Extensive expertise in endocrinology: adrenal crisis. Eur J Endocrinol 2015; 172: R115–R124.
30. Hahner S, Spinnler C, Fassnacht M et al. High incidence of adrenal crisis in educated patients with chronic adrenal insufficiency: a prospective study. J Clin Endocrinol Metab 2015; 100: 407–16.
31. Reisch N, Willige M, Kohn D et al. Frequency and causes of adrenal crises over lifetime in patients with 21-hydroxylase deficiency. Eur J Endocrinol 2012; 167: 35–42.
32. Chortis V, Taylor AE, Schneider P et al. Mitotane therapy in adrenocortical cancer induces CYP3A4 and inhibits 5a-reductase, explaining the need for personalized glucocorticoid and androgen replacement. J Clin Endocrinol Metab 2013; 98: 161–71.
33. Quinkler M, Hahner S. What is the best long-term management strategy for patients with primary adrenal insufficiency? Clin Endocrinol (Oxf) 2012; 76: 21–5.
34. Boonen E, Vervenne H, Meersseman P et al. Reduced cortisol metabolism during critical illness. N Engl J Med 2013; 368: 1477–88.
35. Hahner S, Burger-Stritt S, Allolio B. Subcutaneous hydrocortisone administration for emergency use in adrenal insufficiency. Eur J Endocrinol 2013; 169: 147–54.
36. Hahner S, Hemmelmann N, Quinkler M et al. Timelines in the management of adrenal crisis – targets, limits and reality. Clin Endocrinol (Oxf) 2015; 82: 497–502.
37. Repping-Wuts HJ, Stikkelbroeck NM, Noordzij A et al. A glucocorticoid education group meeting: an effective strategy for improving self-management to prevent adrenal crisis. Eur J Endocrinol 2013; 169: 17–22.
38. Quinkler M, Dahlqvist P, Husebye ES, Kämpe O. A European Emergency Card for adrenal insufficiency can save lives. Eur J Intern Med 2015; 26: 75–6.
39. Reato G, Morlin L, Chen S et al. Premature ovarian failure in patients with autoimmune Addison’s disease: clinical, genetic, and immunological evaluation. J Clin Endocrinol Metab 2011; 96: E1255–E1261.
40. Falorni A, Brozzetti A, Aglietti MC et al. Progressive decline of residual follicle pool after clinical diagnosis of autoimmune ovarian insufficiency. Clin Endocrinol (Oxf) 2012; 77: 453–8.
41. Charmandari E, Nicolaides NC, Chrousos GP. Adrenal insufficiency. Lancet 2014; 383: 2152–67.
42. Christiansen JJ, Bruun JM, Christiansen JS et al. Long-term DHEA substitution in female adrenocortical failure, body composition, muscle function, and bone metabolism: a randomized trial. Eur J Endocrinol 2011; 165: 293–300.
43. Keevil BG. Novel liquid chromatography tandem mass spectrometry (LC-MS/MS) methods for measuring steroids. Best Pract Res Clin Endocrinol Metab 2013; 27: 663–74.
44. Mallappa A, Sinaii N, Kumar P et al. A phase 2 study of Chronocort, a modified-release formulation of hydrocortisone. J Clin Endocrinol Metab 2015; 100: 1137–45.
45. Oksnes M, Björnsdottir S, Isaksson M et al. Continuous subcutaneous hydrocortisone infusion versus oral hydrocortisone replacement for treatment of Addison’s disease: a randomized clinical trial. J Clin Endocrinol Metab 2014; 99: 1665–74.
46. Gagliardi L, Nenke MA, Thynne TR et al. Continuous subcutaneous hydrocortisone infusion therapy in Addison’s disease: a randomized, placebo-controlled clinical trial. J Clin Endocrinol Metab 2014; 99: 4149–57.
47. Russell GM, Durant C, Ataya A et al. Subcutaneous pulsatile glucocorticoid replacement therapy. Clin Endocrinol (Oxf) 2014; 81: 289–93.
48. Gan EH, McArthur K, Mitchell AL et al. Residual adrenal function in autoimmune Addison’s disease: improvement after tetracosactide (ACTH1-24) treatment. J Clin Endocrinol Metab 2014; 99: 111–8.
49. Husebye ES et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014; 275: 104–15.

Г.А.Мельниченко, Е.А.Трошина, М.Ю.Юкина, Н.М.Платонова*, Д.Г.Бельцевич

ФГБУ «Эндокринологический научный центр» Минздрава России. 117036, Россия, Москва, ул. Дмитрия Ульянова, д. 11
*doc-platonova@inbox.ru

________________________________________________

G.A.Melnichenko, E.A.Troshina, M.Yu.Yukina, N.M.Platonova*, D.G.Beltsevicha

Endocrinology research center of the Ministry of Health of the Russian Federation. 117036, Russian Federation, Moscow, ul. Dmitria Ul'ianova, d. 11
*doc-platonova@inbox.ru