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

Simon H S Pearce, Earn H Gan, Catherine Napier, Simon H S Pearce, Earn H Gan, Catherine Napier

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.

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.

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