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Natural history of adrenal steroidogenesis in autoimmune Addison's disease following diagnosis and treatment.

  • Napier, Catherine1
  • Allinson, Kathleen1
  • Gan, Earn H1
  • Mitchell, Anna L1
  • Gilligan, Lorna C2
  • Taylor, Angela E2
  • Arlt, Wiebke2
  • Pearce, Simon H S1
  • 1 Institute of Genetic Medicine, International Centre for Life, Newcastle University, Newcastle upon Tyne, NE1 3BZ; and Newcastle upon Tyne Hospitals NHS Trust, Royal Victoria Infirmary,, UK.
  • 2 Institute of Metabolism and Systems Research (IMSR), University of Birmingham, Birmingham, B15 2TT, UK; and NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, B15 3GW, UK.
Published Article
The Journal of Clinical Endocrinology & Metabolism
The Endocrine Society
Publication Date
Apr 17, 2020
DOI: 10.1210/clinem/dgaa187
PMID: 32300791


The natural history of adrenal function in autoimmune Addison's disease once diagnosed and treated has not been systematically studied, but several case reports of recovery from established adrenal failure suggest it may not be uniform. To ascertain steroidogenic function in autoimmune Addison's disease immediately following diagnosis and during prolonged treatment. We studied peak serum cortisol in response to ACTH1-24 in 20 newly diagnosed autoimmune Addison's disease patients at first presentation and then again within a month. We also studied 37 patients with established Addison's disease (for between 7 months and 44 years) in a medication-free state, measuring peak serum cortisol responses to ACTH1-24 and the urine LC-MS steroid metabolome. Adrenal steroidogenesis declined rapidly after steroid replacement treatment for newly diagnosed Addison's disease was started, with a peak serum cortisol falling from 138 ± 19nmol/l (SEM) at presentation to 63 ± 13nmol/L over 4 weeks (P<0.003). Autoimmune Addison's disease is a heterogeneous condition, showing a rapid decline in adrenal steroidogenesis during the first few weeks following diagnosis, but low-level residual function in a minority of patients, which appears to persist for many years. © Endocrine Society 2020.

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