Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Feb;184(2):R61-R67.
doi: 10.1530/EJE-20-0894.

MANAGEMENT OF ENDOCRINE DISEASE: Residual adrenal function in Addison's disease

Affiliations
Review

MANAGEMENT OF ENDOCRINE DISEASE: Residual adrenal function in Addison's disease

Simon H S Pearce et al. Eur J Endocrinol. 2021 Feb.

Abstract

Over the last 10 years, evidence has accumulated that autoimmune Addison's disease (AAD) is a heterogeneous disease. Residual adrenal function, characterised by persistent secretion of cortisol, other glucocorticoids and mineralocorticoids is present in around 30% of patients with established AAD, and appears commoner in men. This persistent steroidogenesis is present in some patients with AAD for more than 20 years, but it is commoner in people with shorter disease duration. The clinical significance of residual adrenal function is not fully clear at the moment, but as it signifies an intact adrenocortical stem cell population, it opens up the possibility of regeneration of adrenal steroidogenesis and improvement in adrenal failure for some patients.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Urine corticosteroid excretion for 37 patients with autoimmune Addison’s disease in a medication-free state (19). Each patient is represented by the same symbol across each metabolite, including the glucocorticoids: free cortisol and tetrahydro-11-deoxycortisol (THS); and the mineralocorticoids: tetrahydro-11-dehydrocorticosterone (THA) and tetrahydro-corticosterone (THB). Grey bars represent interquartile ranges from healthy individuals.

References

    1. Husebye ES, Allolio B, Arlt W, Badenhoop K, Bensing S, Betterle C, Falorni A, Gan EH, Hulting AL, Kasperlik-Zaluska Aet al. Consensus statement on the diagnosis, treatment and follow-up of patients with primary adrenal insufficiency. Journal of Internal Medicine 2014. 275 104–1. (10.1111/joim.12162) - DOI - PubMed
    1. Løvås K, Husebye ES.High prevalence and increasing incidence of Addison’s disease in western Norway. Clinical Endocrinology 2002. 56 787–7. (10.1046/j.1365-2265.2002.t01-1-01552.x) - DOI - PubMed
    1. Pazderska A, Pearce SH.Adrenal insufficiency: recognition and management. Clinical Medicine 2017. 17 258–262. (10.7861/clinmedicine.17-3-258) - DOI - PMC - PubMed
    1. Winqvist O, Karlsson FA, Kämpe O.21-Hydroxylase, a major autoantigen in idiopathic Addison’s disease. Lancet 1992. 339 1559–15. (10.1016/0140-6736(9291829-w) - DOI - PubMed
    1. Erichsen MM, Løvås K, Skinningsrud B, Wolff AB, Undlien DE, Svartberg J, Fougner KJ, Berg TJ, Bollerslev J, Mella Bet al. Clinical, immunological, and genetic features of autoimmune primary adrenal insufficiency: observations from a Norwegian registry. Journal of Clinical Endocrinology and Metabolism 2009. 94 4882–48. (10.1210/jc.2009-1368) - DOI - PubMed