Adrenal insufficiency overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ayeesha Kattubadi, M.B.B.S[2]

Overview

Historical Perspective

Adrenal insufficiency was first discovered by Thomas Addison in 1849. [1]

Classification

Adrenal insufficiency may be classified according to location of pathology into three subtypes primary adrenal insufficiency, secondary adrenal insufficiency, and tertiary adrenal insufficiency. [2]

Pathophysiology

It is thought that adrenal insufficiency is the result of multiple etiologies like autoimmune adrenalitis, X-linked adrenoleukodystrophy, chronic glucocorticoid use, adrenal hemoorage etc. The pathophysiology depends on each etiological subtype. [3] [4][5] [6][7] [8]

Causes

Common causes of adrenal insufficiency include autoimmune adrenalitis and chronic glucocorticoid use. [2] [9]

Differentiating adrenal insufficiency from Other Diseases

Addison's disease must be differentiated from other diseases that cause hypotension, skin pigmentation, and abdominal pain such as myopathies, celiac disease, Peutz-Jeghers syndrome, anorexia nervosa, syndrome of inappropriate antidiuretic hormone (SIADH), neurofibromatosis, porphyria cutanea tarda, salt-depletion nephritis and bronchogenic carcinoma.

Epidemiology and Demographics

Adrenal insufficiency is a rare disorder. It may present in patients of all age groups. The incidence of autoimmune adrenalitis is more common in females in the age groups of 30 to 50 compared to males. Whereas, adrenal crisis does not show any female to male predilection. The most common cause of primary adrenal insufficiency in the developed world is autoimmune adrenalitis. Whereas, in the developing world it is Tuberculosis. The most common cause of central adrenal insufficiency is chronic glucocorticoid use. [10] [11]

Risk Factors

Common risk factors in the development of adrenal insufficiency include chronic glucocorticoid use, tuberculosis, anticoagulant use, etc. [12].

Screening

There is insufficient evidence to recommend routine screening for adrenal insufficiency. [13]

Natural History, Complications, and Prognosis

Common complications of adrenal insufficiency include cardiovascular complications, disturbed sleep, reduced bone mineral density. Prognosis is generally excellent with replacement therapy. [14][4]

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

References

  1. BISHOP PM (1950). "The history of the discovery of Addison's disease". Proc R Soc Med. 43 (1): 35–42. PMC 2081266. PMID 15409948.
  2. 2.0 2.1 Feingold KR, Anawalt B, Boyce A, Chrousos G, de Herder WW, Dungan K; et al. (2000). "Endotext". PMID 25905309.
  3. "StatPearls". 2020. PMID 28722862.
  4. 4.0 4.1 Erichsen, Martina M.; Løvås, Kristian; Skinningsrud, Beate; Wolff, Anette B.; Undlien, Dag E.; Svartberg, Johan; Fougner, Kristian J.; Berg, Tore J.; Bollerslev, Jens; Mella, Bjarne; Carlson, Joyce A.; Erlich, Henry; Husebye, Eystein S. (2009). "Clinical, Immunological, and Genetic Features of Autoimmune Primary Adrenal Insufficiency: Observations from a Norwegian Registry". The Journal of Clinical Endocrinology & Metabolism. 94 (12): 4882–4890. doi:10.1210/jc.2009-1368. ISSN 0021-972X.
  5. Huffnagel, Irene C; Laheji, Fiza K; Aziz-Bose, Razina; Tritos, Nicholas A; Marino, Rose; Linthorst, Gabor E; Kemp, Stephan; Engelen, Marc; Eichler, Florian (2019). "The Natural History of Adrenal Insufficiency in X-Linked Adrenoleukodystrophy: An International Collaboration". The Journal of Clinical Endocrinology & Metabolism. 104 (1): 118–126. doi:10.1210/jc.2018-01307. ISSN 0021-972X.
  6. Berger J, Forss-Petter S, Eichler FS (2014). "Pathophysiology of X-linked adrenoleukodystrophy". Biochimie. 98: 135–42. doi:10.1016/j.biochi.2013.11.023. PMC 3988840. PMID 24316281.
  7. Feingold KR, Anawalt B, Boyce A, Chrousos G, de Herder WW, Dungan K; et al. (2000). "Endotext". PMID 25905379.
  8. Younes, Areej K.; Younes, Noor K. (2017). "Recovery of steroid induced adrenal insufficiency". Translational Pediatrics. 6 (4): 269–273. doi:10.21037/tp.2017.10.01. ISSN 2224-4336.
  9. Bornstein, Stefan R.; Allolio, Bruno; Arlt, Wiebke; Barthel, Andreas; Don-Wauchope, Andrew; Hammer, Gary D.; Husebye, Eystein S.; Merke, Deborah P.; Murad, M. Hassan; Stratakis, Constantine A.; Torpy, David J. (2016). "Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline". The Journal of Clinical Endocrinology & Metabolism. 101 (2): 364–389. doi:10.1210/jc.2015-1710. ISSN 0021-972X.
  10. Melmed, Shlomo (2020). Williams textbook of endocrinology. Philadelphia, PA: Elsevier. ISBN 9780323555968.
  11. Elshimy G, Alghoula F, Jeong JM. PMID 29763143. Missing or empty |title= (help)
  12. "www.amjmed.com".
  13. Chanson, Philippe; Guignat, Laurence; Goichot, Bernard; Chabre, Olivier; Boustani, Dinane Samara; Reynaud, Rachel; Simon, Dominique; Tabarin, Antoine; Gruson, Damien; Reznik, Yves; Raffin Sanson, Marie-Laure (2017). "Group 2: Adrenal insufficiency: screening methods and confirmation of diagnosis". Annales d'Endocrinologie. 78 (6): 495–511. doi:10.1016/j.ando.2017.10.005. ISSN 0003-4266.
  14. "www.amjmed.com".


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