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

Lookup NU author(s): Dr Catherine Napier, Kathleen Allinson, Dr Earn Gan, Dr Anna Mitchell, Professor Simon PearceORCiD

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This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).

Abstract

Context: 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. Objective: To ascertain steroidogenic function in autoimmune Addison’s disease immediately following diagnosis and during prolonged treatment. Design: 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. Results: 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). Six of 37 participants (16%) with established Addison’s disease had detectable serum cortisol and urine glucocorticoid and mineralocorticoid metabolites during repeat testing, indicating variable degrees of residual adrenal function. Conclusion: 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.

Publication metadata

Author(s): Napier C, Allinson K, Gan EH, Mitchell AL, Gilligan LC, Taylor AE, Arlt W, Pearce SHS

Publication type: Article

Publication status: Published

Journal: Journal of Clinical Endocrinology & Metabolism

Year: 2020

Volume: 105

Issue: 7

Pages: 2322-2330

Print publication date: 01/07/2020

Online publication date: 17/04/2020

Acceptance date: 09/04/2020

Date deposited: 04/05/2020

ISSN (print): 0021-972X

ISSN (electronic): 1945-7197

Publisher: Oxford University Press

URL: https://doi.org/10.1210/clinem/dgaa187

DOI: 10.1210/clinem/dgaa187

PubMed id: 32300791

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