Adrenal insufficiency: Review of clinical outcomes with current glucocorticoid replacement therapy

Clinical Endocrinology

Volumn 82, Issue 1, 2015, Pages 2-11

  Johannsson, Gudmundur ^a   Falorni, Alberto ^b   Skrtic, Stanko ^a,c   Lennernäs, Hans ^d   Quinkler, Marcus ^e   Monson, John P ^f   Stewart, Paul M ^g  

^a UNIVERSITY OF GOTHENBURG   (Sweden)

^b UNIVERSITY OF PERUGIA   (Italy)

^c ASTRAZENECA R AND D   (Sweden)

^d UPPSALA UNIVERSITY   (Sweden)

^e Endocrinology in Charlottenburg   (Germany)

^f QUEEN MARY UNIVERSITY OF LONDON   (United Kingdom)

^g UNIVERSITY OF LEEDS   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

CORTISONE ACETATE; GLUCOCORTICOID; GLUCOSE; HYDROCORTISONE; LIPID; PREDNISOLONE; PREDNISONE;

ADRENAL CORTEX INSUFFICIENCY; ADRENAL INSUFFICIENCY; BLOOD PRESSURE; BODY COMPOSITION; BONE METABOLISM; CARDIOVASCULAR FUNCTION; CORTICOSTEROID THERAPY; GLUCOSE METABOLISM; HUMAN; LIPID METABOLISM; MORTALITY; NONHUMAN; PRIMARY ADRENAL INSUFFICIENCY; PRIORITY JOURNAL; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; SECONDARY ADRENAL INSUFFICIENCY; UNSPECIFIED SIDE EFFECT; ADVERSE EFFECTS; HORMONE SUBSTITUTION; PROCEDURES; TREATMENT OUTCOME;

ADRENAL INSUFFICIENCY; GLUCOCORTICOIDS; HORMONE REPLACEMENT THERAPY; HUMANS; TREATMENT OUTCOME;

EID: 84928788801     PISSN: 03000664     EISSN: 13652265     Source Type: Journal    
DOI: 10.1111/cen.12603     Document Type: Review

References (79)

1. Bishop, P.M.F. (1950) The history of the discovery of Addison's disease. Proceedings of the Royal Society of Medicine, 43, 35-42.
2. Dunlop, D. (1963) Eighty-six cases of Addison's disease. British Medical Journal, 2, 887-891.
3. Hillier, S.G. (2007) Diamonds are forever: The cortisone legacy. Journal of Endocrinology, 195, 1-6.
4. Cope, C.L. & Black, E. (1958) The production rate of cortisol in man. British Medical Journal, 2, 887-891.
5. Derendorf, H., Mollmann, H., Barth, J. et al. (1991) Pharmacokinetics and oral bioavailability of hydrocortisone. Journal of Clinical Pharmacology, 31, 473-476.
6. Johannsson, G., Nilsson, A.G., Bergthorsdottir, R. et al. (2012) Improved cortisol exposure-time profile and outcome in patients with adrenal insufficiency: a prospective randomized trial of a novel hydrocortisone dual-release formulation. Journal of Clinical Endocrinology and Metabolism, 97, 473-481.
7. Simon, N., Castinetti, F., Ouliac, F. et al. (2010) Pharmacokinetic evidence for suboptimal treatment of adrenal insufficiency with currently available hydrocortisone tablets. Clinical Pharmacokinetics, 49, 455-463.
8. Blomgren, J., Ekman, B., Andersson, P.O. et al. (2004) Nonphysiological levels of circulating cortisol in growth hormonetreated hypopituitary adults after conventional cortisone substitution. Scandinavian Journal of Clinical and Laboratory Investigation, 64, 132-139.
9. Krieger, D.T., Allen, W., Rizzo, F. et al. (1971) Characterization of the normal temporal pattern of plasma corticosteroid levels. Journal of Clinical Endocrinology and Metabolism, 32, 266-284.
10. Bensing, S., Brandt, L., Tabaroj, F. et al. (2008) Increased death risk and altered cancer incidence pattern in patients with isolated or combined autoimmune primary adrenocortical insufficiency. Clinical Endocrinology (Oxford), 69, 697-704.
11. Bergthorsdottir, R., Leonsson-Zachrisson, M., Oden, A. et al. (2006) Premature mortality in patients with Addison's disease: a population-based study. Journal of Clinical Endocrinology and Metabolism, 91, 4849-4853.
12. Rosen, T. & Bengtsson, B.A. (1990) Premature mortality due to cardiovascular disease in hypopituitarism. Lancet, 336, 285-288.
13. Tomlinson, J.W., Holden, N., Hills, R.K. et al. (2001) Association between premature mortality and hypopituitarism. West Midlands Prospective Hypopituitary Study Group. Lancet, 357, 425-431.
14. Mason, A.S., Meade, T.W., Lee, J.A. et al. (1968) Epidemiological and clinical picture of Addison's disease. Lancet, 2, 744-747.
15. Crown, A. & Lightman, S. (2005) Why is the management of glucocorticoid deficiency still controversial: a review of the literature. Clinical Endocrinology, 63, 483-492.
16. Arlt, W. & Allolio, B. (2003) Adrenal insufficiency. Lancet, 361, 1881-1893.
17. Løvas, K. & Husebye, E.S. (2002) High prevalence and increasing incidence of Addison's disease in western Norway. Clinical Endocrinology, 56, 787-791.
18. Erichsen, M.M., Løvas, K., Fougner, K.J. et al. (2009) Normal overall mortality rate in Addison's disease, but young patients are at risk of premature death. European Journal of Endocrinology, 160, 233-237.
19. Gaillard, R.C., Mattsson, A.F., Akerblad, A.C. et al. (2012) Overall and cause-specific mortality in GH-deficient adults on GH replacement. European Journal of Endocrinology, 166, 1069-1077.
20. Burman, P., Mattsson, A.F., Johannsson, G. et al. (2013) Deaths among adult patients with hypopituitarism: hypocortisolism during acute stress, and de novo malignant brain tumors contribute to an increased mortality. Journal of Clinical Endocrinology and Metabolism, 98, 1466-1475.
21. Mills, J.L., Schonberger, L.B., Wysowski, D.K. et al. (2004) Longterm mortality in the United States cohort of pituitary-derived growth hormone recipients. Journal of Pediatrics, 144, 430-436.
22. Sherlock, M., Reulen, R.C., Alonso, A.A. et al. (2009) ACTH deficiency, higher doses of hydrocortisone replacement, and radiotherapy are independent predictors of mortality in patients with acromegaly. Journal of Clinical Endocrinology and Metabolism, 94, 4216-4223.
23. Hahner, S., Loeffler, M., Bleicken, B. et al. (2010) Epidemiology of adrenal crisis in chronic adrenal insufficiency: The need for new prevention strategies. European Journal of Endocrinology, 162, 597-602.
24. White, K. & Arlt, W. (2010) Adrenal crisis in treated Addison's disease: a predictable but under-managed event. European Journal of Endocrinology, 162, 115-120.
25. Omori, K., Nomura, K., Shimizu, S. et al. (2003) Risk factors for adrenal crisis in patients with adrenal insufficiency. Endocrine Journal, 50, 745-752.
26. Leblicq, C., Rottembourg, D., Deladoey, J. et al. (2011) Are guidelines for glucocorticoid coverage in adrenal insufficiency currently followed? Journal of Pediatrics, 158, 492-498.
27. Reisch, N., Willige, M., Kohn, D. et al. (2012) Frequency and causes of adrenal crises over lifetime in patients with 21-hydroxylase deficiency. European Journal of Endocrinology, 167, 35-42.
28. Munck, A. & Naray-Fejes-Toth, A. (2006) Glucocorticoid physiology. In: L.J. DeGrooth, J.C. Jameson eds. Endocrinology. Elsevier Saunders, Philadelphia, Pennsylvania, 2287-2309.
29. Canalis, E. & Giustina, A. (2001) Glucocorticoid-induced osteoporosis: summary of a workshop. Journal of Clinical Endocrinology and Metabolism, 86, 5681-5685.
30. Arlt, W., Rosenthal, C., Hahner, S. et al. (2006) Quality of glucocorticoid replacement in adrenal insufficiency: clinical assessment vs. timed serum cortisol measurements. Clinical Endocrinology, 64, 384-389.
31. Jodar, E., Valdepenas, M.P., Martinez, G. et al. (2003) Long-term follow-up of bone mineral density in Addison's disease. Clinical Endocrinology, 58, 617-620.
32. Valero, M.A., Leon, M., Ruiz Valdepenas, M.P. et al. (1994) Bone density and turnover in Addison's disease: effect of glucocorticoid treatment. Bone and Mineral, 26, 9-17.
33. Devogelaer, J.P., Crabbe, J. & Nagant de Deuxchaisnes, C. (1987) Bone mineral density in Addison's disease: evidence for an effect of adrenal androgens on bone mass. British Medical Journal (Clinical Research Ed.), 294, 798-800.
34. Florkowski, C.M., Holmes, S.J., Elliot, J.R. et al. (1994) Bone mineral density is reduced in female but not male subjects with Addison's disease. The New Zealand Medical Journal, 107, 52-53.
35. Braatvedt, G.D., Joyce, M., Evans, M. et al. (1999) Bone mineral density in patients with treated Addison's disease. Osteoporosis International, 10, 435-440.
36. Koetz, K.R., Ventz, M., Diederich, S. et al. (2012) Bone mineral density is not significantly reduced in adult patients on low-dose glucocorticoid replacement therapy. Journal of Clinical Endocrinology and Metabolism, 97, 85-92.
37. Zelissen, P.M., Croughs, R.J., van Rijk, P.P. et al. (1994) Effect of glucocorticoid replacement therapy on bone mineral density in patients with Addison disease. Annals of Internal Medicine, 120, 207-210.
38. Leelarathna, L., Breen, L., Powrie, J.K. et al. (2010) Co-morbidities, management and clinical outcome of auto-immune Addison's disease. Endocrine, 38, 113-117.
39. Løvas, K., Gjesdal, C.G., Christensen, M. et al. (2009) Glucocorticoid replacement therapy and pharmacogenetics in Addison's disease: effects on bone. European Journal of Endocrinology, 160, 993-1002.
40. Bjornsdottir, S., Saaf, M., Bensing, S. et al. (2011) Risk of hip fracture in Addison's disease: a population-based cohort study. Journal of Internal Medicine, 270, 187-195.
41. Holmes, S.J., Economou, G., Whitehouse, R.W. et al. (1994) Reduced bone mineral density in patients with adult onset growth hormone deficiency. Journal of Clinical Endocrinology and Metabolism, 78, 669-674.
42. Wuster, C., Abs, R., Bengtsson, B.A. et al. (2001) The influence of growth hormone deficiency, growth hormone replacement therapy, and other aspects of hypopituitarism on fracture rate and bone mineral density. Journal of Bone and Mineral Research, 16, 398-405.
43. Ragnarsson, O., Nystrom, H.F. & Johannsson, G. (2012) Glucocorticoid replacement therapy is independently associated with reduced bone mineral density in women with hypopituitarism. Clinical Endocrinology, 76, 246-252.
44. Holmer, H., Svensson, J., Rylander, L. et al. (2007) Fracture incidence in GH-deficient patients on complete hormone replacement including GH. Journal of Bone and Mineral Research, 22, 1842-1850.
45. Axelrod, L. (1976) Glucocorticoid therapy. Medicine (Baltimore), 55, 39-65.
46. Walker, B.R. (2007) Glucocorticoids and cardiovascular disease. European Journal of Endocrinology, 157, 545-559.
47. Wei, L., MacDonald, T.M. & Walker, B.R. (2004) Taking glucocorticoids by prescription is associated with subsequent cardiovascular disease. Annals of Internal Medicine, 141, 764-770.
48. Markussis, V., Beshyah, S.A., Fisher, C. et al. (1992) Detection of premature atherosclerosis by high-resolution ultrasonography in symptom-free hypopituitary adults. Lancet, 340, 1188-1192.
49. Wilsson J.D., Foster D.W., Kronenberg H.M. et al. (eds) (2002) Williams Textbook of Endocrinology, 10th edn. W.B. Saunders Company, London.
50. Lightman, S.L., Wiles, C.C., Atkinson, H.C. et al. (2008) The significance of glucocorticoid pulsatility. European Journal of Pharmacology, 583, 255-262.
51. Dallman, M.F., Akana, S.F., Bhatnagar, S. et al. (2000) Bottomed out: metabolic significance of the circadian trough in glucocorticoid concentrations. International Journal of Obesity and Related Metabolic Disorders, 24(Suppl 2), S40-S46.
52. Verhelst, J., Mattsson, A.F., Luger, A. et al. (2011) Prevalence and characteristics of the metabolic syndrome in 2479 hypopituitary patients with adult-onset GH deficiency before GH replacement: a KIMS analysis. European Journal of Endocrinology, 165, 881-889.
53. Abs, R., Mattsson, A.F., Thunander, M. et al. (2013) Prevalence of diabetes mellitus in 6050 hypopituitary patients with adultonset GH deficiency before GH replacement: a KIMS analysis. European Journal of Endocrinology, 168, 297-305.
54. Giordano, R., Marzotti, S., Balbo, M. et al. (2009) Metabolic and cardiovascular profile in patients with Addison's disease under conventional glucocorticoid replacement. Journal of Endocrinological Investigation, 32, 917-923.
55. Ross, I.L., Bergthorsdottir, R., Levitt, N.S. et al. (2013) Increased cardiovascular risk in south african patients with Addison's disease. Hormone and Metabolic Research, 45, 905-910.
56. Filipsson, H., Monson, J.P., Koltowska-Haggstrom, M. et al. (2006) The impact of glucocorticoid replacement regimens on metabolic outcome and comorbidity in hypopituitary patients. Journal of Clinical Endocrinology and Metabolism, 91, 3954-3961.
57. Al-Shoumer, K.A., Beshyah, S.A., Niththyananthan, R. et al. (1995) Effect of glucocorticoid replacement therapy on glucose tolerance and intermediary metabolites in hypopituitary adults. Clinical Endocrinology, 42, 85-90.
58. Elbelt, U., Hahner, S. & Allolio, B. (2009) Altered insulin requirement in patients with type 1 diabetes and primary adrenal insufficiency receiving standard glucocorticoid replacement therapy. European Journal of Endocrinology, 160, 919-924.
59. Plat, L., Leproult, R., L'Hermite-Baleriaux, M. et al. (1999) Metabolic effects of short-term elevations of plasma cortisol are more pronounced in the evening than in the morning. Journal of Clinical Endocrinology and Metabolism, 84, 3082-3092.
60. Danilowicz, K., Bruno, O.D., Manavela, M. et al. (2008) Correction of cortisol overreplacement ameliorates morbidities in patients with hypopituitarism: a pilot study. Pituitary, 11, 279- 285.
61. Dunne, F.P., Elliot, P., Gammage, M.D. et al. (1995) Cardiovascular function and glucocorticoid replacement in patients with hypopituitarism. Clinical Endocrinology, 43, 623-629.
62. Peacey, S.R., Guo, C.Y., Robinson, A.M. et al. (1997) Glucocorticoid replacement therapy: are patients over treated and does it matter? Clinical Endocrinology, 46, 255-261.
63. Løvas, K., Loge, J.H. & Husebye, E.S. (2002) Subjective health status in Norwegian patients with Addison's disease. Clinical Endocrinology, 56, 581-588.
64. Thomsen, A.F., Kvist, T.K., Andersen, P.K. et al. (2006) The risk of affective disorders in patients with adrenocortical insufficiency. Psychoneuroendocrinology, 31, 614-622.
65. Hahner, S., Loeffler, M., Fassnacht, M. et al. (2007) Impaired subjective health status in 256 patients with adrenal insufficiency on standard therapy based on cross-sectional analysis. Journal of Clinical Endocrinology and Metabolism, 92, 3912-3922.
66. Bleicken, B., Hahner, S., Ventz, M. et al. (2010) Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients. American Journal of the Medical Sciences, 339, 525- 531.
67. Forss, M., Batcheller, G., Skrtic, S. et al. (2012) Current practice of glucocorticoid replacement therapy and patient-perceived health outcomes in adrenal insufficiency - A worldwide patient survey. BMC Endocrine Disorders, 12, 8.
68. Tiemensma, J., Andela, C.D., Kaptein, A.A. et al. (2014) Psychological morbidity and impaired quality of life in patients with stable treatment for primary adrenal insufficiency: cross-sectional study and review of the literature. European Journal of Endocrinology, 171, 171-182.
69. Ragnarsson, O., Mattsson, A.F., Monson, J.P. et al. (2014) The relationship between glucocorticoid replacement and quality of life in 2737 hypopituitary patients. European Journal of Endocrinology (Epub ahead of print).
70. Falorni, A., Minarelli, V. & Morelli, S. (2013) Therapy of adrenal insufficiency: an update. Endocrine, 43, 514-528.
71. Bleicken, B., Hahner, S., Loeffler, M. et al. (2008) Impaired subjective health status in chronic adrenal insufficiency: impact of different glucocorticoid replacement regimens. European Journal of Endocrinology, 159, 811-817.
72. Bleicken, B., Hahner, S., Loeffler, M. et al. (2010) Influence of hydrocortisone dosage scheme on health-related quality of life in patients with adrenal insufficiency. Clinical Endocrinology, 72, 297-304.
73. Alonso, N., Granada, M.L., Lucas, A. et al. (2004) Evaluation of two replacement regimens in primary adrenal insufficiency patients. effect on clinical symptoms, health-related quality of life and biochemical parameters. Journal of Endocrinological Investigation, 27, 449-454.
74. Riedel, M., Wiese, A., Schurmeyer, T.H. et al. (1993) Quality of life in patients with Addison's disease: effects of different cortisol replacement modes. Experimental and Clinical Endocrinology, 101, 106-111.
75. Groves, R.W., Toms, G.C., Houghton, B.J. et al. (1988) Corticosteroid replacement therapy: Twice or thrice daily? Journal of the Royal Society of Medicine, 81, 514-516.
76. Wichers, M., Springer, W., Bidlingmaier, F. et al. (1999) The influence of hydrocortisone substitution on the quality of life and parameters of bone metabolism in patients with secondary hypocortisolism. Clinical Endocrinology (Oxford), 50, 759-765.
77. Behan, L.A., Rogers, B., Hannon, M.J. et al. (2011) Optimizing glucocorticoid replacement therapy in severely adrenocorticotropin- deficient hypopituitary male patients. Clinical Endocrinology, 75, 505-513.
78. Ekman, B., Bachrach-Lindstrom, M., Lindstrom, T. et al. (2012) A randomized, double-blind, crossover study comparing twoand four-dose hydrocortisone regimen with regard to quality of life, cortisol and ACTH profiles in patients with primary adrenal insufficiency. Clinical Endocrinology, 77, 18-25.
79. Benson, S., Neumann, P., Unger, N. et al. (2012) Effects of standard glucocorticoid replacement therapies on subjective wellbeing: a randomized, double-blind, crossover study in patients with secondary adrenal insufficiency. European Journal of Endocrinology, 167, 679-685.