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Addison’s Disease

  • Living reference work entry
  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

  • 12 Accesses

Synonyms

Primary adrenal insufficiency

Definition

Addison’s disease is a chronic deficiency of glucocorticoids and/or mineralocorticoids secretion due to adrenal cortex insufficiency.

Clinical Features

  • Incidence and Prevalence

    Addison’s disease is rare with an estimated annual incidence of four to six cases per million people. The prevalence in Western countries has increased over the last 60 years and is now estimated to be 100–140 cases per million people. This observed increase is probably due to the improvements in the diagnostic ability to detect the disease (Erichsen et al. 2009; Laureti et al. 1999; Meyer et al. 2014; Olafsson and Sigurjonsdottir 2016).

  • Age

    Patients of all ages can be affected, but the disease presents more frequently between the second and fourth decade of life.

  • Sex

    Women are more frequently affected than men.

  • Site

    Adrenal gland.

  • Etiology

    Etiological factors causing Addison’s disease have changed over the last decades. While in the past the disease was more...

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References and Further Reading

  • Baker, P. R., Baschal, E. E., Fain, P. R., et al. (2010). Haplotype analysis discriminates genetic risk for DR3-associated endocrine autoimmunity and helps define extreme risk for Addison’s disease. Journal of Clinical Endocrinology and Metabolism, 95, E263–E270.

    Article  CAS  Google Scholar 

  • Barthel, A., Benker, G., Berens, K., et al. (2019). An update on Addison’s disease. Experimental and Clinical Endocrinology and Diabetes, 127, 165–175.

    Article  CAS  Google Scholar 

  • Bornstein, S. R., Allolio, B., Arlt, W., et al. (2016). Diagnosis and treatment of primary adrenal insufficiency: An endocrine society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism, 101, 364–389.

    Article  CAS  Google Scholar 

  • Erichsen, M. M., Løvås, K., Fougner, K. J., Svartberg, J., Hauge, E. R., Bollerslev, J., Berg, J. P., Mella, B., & Husebye, E. S. (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.

    Article  CAS  Google Scholar 

  • Eriksson, D., Bianchi, M., Landegren, N., et al. (2016). Extended exome sequencing identifies BACH2 as a novel major risk locus for Addison’s disease. Journal of Internal Medicine, 280, 595–608.

    Article  CAS  Google Scholar 

  • Hellesen, A., Bratland, E., & Husebye, E. S. (2018 Jun). Autoimmune Addison’s disease – An update on pathogenesis. Annales d’Endocrinologie, 79(3), 157–163.

    Article  Google Scholar 

  • Husebye, E. S., Anderson, M. S., & Kämpe, O. (2018). Autoimmune polyendocrine syndromes. New England Journal of Medicine, 378, 1132–1141.

    Article  CAS  Google Scholar 

  • Laureti, S., Vecchi, L., Santeusanio, F., et al. (1999). Is the prevalence of Addison’s disease underestimated? Journal of Clinical Endocrinology and Metabolism, 84, 1762.

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  • Lebbe, M., & Arlt, W. (2013). What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy? Clinical Endocrinology, 78, 497–502.

    Article  CAS  Google Scholar 

  • Meyer, G., Neumann, K., Badenhoop, K., et al. (2014). Increasing prevalence of Addison’s disease in German females: Health insurance data 2008–2012. European Journal of Endocrinology, 170, 367–373.

    Article  CAS  Google Scholar 

  • Mitchell, A. L., & Pearce, S. H. (2012). Autoimmune Addison disease: Pathophysiology and genetic complexity. Nature Reviews. Endocrinology, 8, 306–316.

    Article  CAS  Google Scholar 

  • Olafsson, A. S., & Sigurjonsdottir, H. A. (2016). Increasing prevalence of Addison disease: Results from a nationwide study. Endocrine Practice, 22, 30–35.

    Article  Google Scholar 

  • Quinkler, M., Oelkers, W., Remde, H., et al. (2015). Mineralocorticoid substitution and monitoring in primary adrenal insufficiency. Best Practice and Research. Clinical Endocrinology and Metabolism, 29, 17–24.

    Article  CAS  Google Scholar 

  • Winqvist, O., Karlsson, F. A., & Kampe, O. (1992). 21-Hydroxylase, a major autoantigen in idiopathic Addison’s disease. Lancet, 339, 1559–1562.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Pathology Unit, Department of Medicine and Surgery, University of Insubria, Varese, Italy

    Stefano La Rosa

Authors
  1. Stefano La Rosa
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Corresponding author

Correspondence to Stefano La Rosa .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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La Rosa, S. (2022). Addison’s Disease. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5243-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_5243-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

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