Chronic adrenal insufficiency in clinical practice

Cover Page

Cite item

Full Text

Abstract

Aim. To present causes, pathogenesis, clinical presentation, diagnostics and treatment of chronic adrenal insufficiency. Materials and methods. Data from 23 scientific sources published in Russia and abroad in years 1985-2017 are presented. Results. Although adrenal insufficiency is a rare endocrine system disorder, early diagnostics and treatment of adrenal insufficiency (AI) is of great importance in clinical practice because this is a life-threatening disorder when not treated. Depending on the level of hypothalamic-pituitary-adrenal system damage, primary (PAI), secondary (SAI) and tertiary adrenal insufficiency are distinguished. The main cause of PAI (80-90%) is an autoimmune destruction of adrenal cortex (autoimmune adrenalitis): isolated damage or a part of autoimmune polyglandular syndrome. Adrenal glands damage can also develop as a result of various factors: infections (tuberculosis, fungous and cytomegalovirus infections), tumors (malignant tumors or metastases), adrenalectomy, and adrenoleukodystrophy. The most frequent cause of SAI is glucocorticoids therapy in pharmacological doses, timorous and destructive processes in pituitary-hypothalamic area that result in ACTH production decrease and suppression. Clinical presentation is not a highly specific criterion of CAI diagnostics and requires confirmation with laboratory results. AI clinical manifestations aggravate in association with stressful situations, intercurrent diseases, high fever, and surgical interventions. A patient with chronic primary adrenal insufficiency needs constant replacement therapy with glucocorticoids and, in most cases with mineralocorticoids. Conclusion. Awareness of AI clinical presentation and modern diagnostic methods will allow diagnosing the disease on time and prescribing replacement therapy.

About the authors

Elena V. Biryukova

A.I.Evdokimov Moscow State University of Medicine and Dentistry; A.S.Loginov Moscow Clinical Scientific Practical Center

Email: *lena@obsudim.ru
д-р мед. наук, проф. каф. эндокринологии и диабетологии Moscow, Russia

Ekaterina S. Ganenkova

A.I.Evdokimov Moscow State University of Medicine and Dentistry

Email: E_ganenkova@bk.ru
Clinical Resident Moscow, Russia

Marina D. Lovanova

A.I.Evdokimov Moscow State University of Medicine and Dentistry

Email: lovanovam@bk.ru
Clinical Resident Moscow, Russia

References

  1. Bleicken B, Hahner S, Ventz M, Quinkler M. Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients. Am J Med Sci 2010; 339: 525-31.
  2. Husebye E.S, Allolio B, Arlt W et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014; 275: 104-15.
  3. Pazderska A, Pearce S.H.S. Adrenal insufficiency - recognition and management. Clin Med 2017; 17 (3): 258-62.
  4. Betterle C, Scalici C, Presotto F et al. The natural history of adrenal function in autoimmune patients with adrenal autoantibodies. J Endocrinol 1988; 117 (3): 467-75.
  5. Клиническая эндокринология: рук. 3-е изд. Под ред. Н.Т Старковой. СПб: Питер, 2002; с. 305-12.
  6. Arlt W, Allolio B. Adrenal insufficiency. Lancet 2003; 361: 1881-93.
  7. Laureti S, Vecchi L, Santeusanio F, Falorni A. Is the prevalence of Addison’s disease underestimated? J Clin Endocrinol Metab 1999; 84 (5): 1762.
  8. Kong M.F, Jeffocoate W. Eighty-six cases of Addison’s disease. Clin Endocrinol (Oxf) 1994; 41 (6): 757-61.
  9. Regal M, Páramo C, Sierra S.M, Garcia-Mayor R.V. Prevalence and incidence of hypopituitarism in an adult Caucasian population in northwestern Spain. Clin Endocrinol 2001; 55: 735-40.
  10. Husebye E.S, Løvås K. Immunology of Addison's disease and premature ovarian failure. Endocrinol Metab Clin North Am 2009; 38: 389.
  11. Bleicken B, Hahner S, Ventz M, Quinkler M. Delayed diagnosis of adrenal insufficiency is common: a cross-sectional study in 216 patients. Am J Med Sci 2010; 339 (6): 525-31.
  12. Eisenbarth G.S, Gottlieb P.A. Autoimmune polyendocrine syndromes. N Engl J Med 2004; 350: 2068-79.
  13. Winqvist O, Karlsson F.A, Kampe O. 21-Hydroxylase, a major autoantigen in idiopathic Addison’s disease. Lancet 1992; 339: 1559-62.
  14. Eledrisi M.S, Verghese A.C. Adrenal insufficiency in HIV Infection: A review and recommendations. Am J Med Sci 2001; 321 (2): 137-44.
  15. Engelen M, Kemp S, de Visser M et al. X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management. Orphanet J Dis 2012; 7: 51-8.
  16. Борисова Е.О. Побочные эффекты системной глюкокортикостероидной терапии. Пульмонология и аллергология. 2004; 3: 14-8.
  17. Gilchrist F.J, Cox K.J, Rowe R et al. Itraconazole and inhaled fluti-casone causing hypothalamic-pituitary-adrenal axis suppression in adults with cystic fibrosis. J Cyst Fibros 2013; 12: 399-402.
  18. Préville-Ratelle S, Coriati A, Ménard A. Adrenal Insufficiency in Cystic Fibrosis: A Rare Phenomenon? Can Resp J 2018; Article ID 3629031. https://doi.org/10.1155/2018/3629031
  19. Løvås K, Husebye E.S. Addison's disease. Lancet 2005; 365: 2058-61.
  20. Husebye E, Løvås K. Pathogenesis of primary adrenal insufficiency. Best Pract Res Clin Endocrinol Metab 2009; 23: 147-57.
  21. Husebye E.S, Allolio B, Arlt W et al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. J Intern Med 2014; 275: 104-15.
  22. Stryker T.D, Molitch M.E. Reversible hyperthyrotropinemia, hyperthyroxinemia, and hyperprolactinemia due to adrenalinsufficiency. Am J Med 1985; 79: 271-6.
  23. Bornstein S.R, Allolio B, Arlt W. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 2016; 101 (2): 364-89.

Copyright (c) 2019 Consilium Medicum

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies