Natural history of adrenal steroidogenesis in autoimmune Addison's disease following diagnosis and treatment
File(s)
Published version
Author(s)
Type
Journal Article
Abstract
Context
The natural history of adrenal function in autoimmune Addison 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 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 disease patients at first presentation and then again within a month. We also studied 37 patients with established Addison 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 disease was started, with a peak serum cortisol falling from 138 ± 19 nmol/L (SEM) at presentation to 63 ± 13 nmol/L over 4 weeks (P < 0.003).
Six of 37 participants (16%) with established Addison disease had detectable serum cortisol and urine glucocorticoid and mineralocorticoid metabolites during repeat testing, indicating variable degrees of residual adrenal function.
Conclusion
Autoimmune Addison 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.
The natural history of adrenal function in autoimmune Addison 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 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 disease patients at first presentation and then again within a month. We also studied 37 patients with established Addison 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 disease was started, with a peak serum cortisol falling from 138 ± 19 nmol/L (SEM) at presentation to 63 ± 13 nmol/L over 4 weeks (P < 0.003).
Six of 37 participants (16%) with established Addison disease had detectable serum cortisol and urine glucocorticoid and mineralocorticoid metabolites during repeat testing, indicating variable degrees of residual adrenal function.
Conclusion
Autoimmune Addison 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.
Date Issued
2020-07-01
Date Acceptance
2020-04-15
Citation
Journal of Clinical Endocrinology and Metabolism (JCEM), 2020, 105 (7), pp.2322-2330
ISSN
0021-972X
Publisher
Oxford University Press
Start Page
2322
End Page
2330
Journal / Book Title
Journal of Clinical Endocrinology and Metabolism (JCEM)
Volume
105
Issue
7
Copyright Statement
© Endocrine Society 2020. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits un restricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
License URL
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/32300791
PII: 5821191
Subjects
Endocrinology & Metabolism
INSUFFICIENCY
Life Sciences & Biomedicine
RECOVERY
Science & Technology
THERAPY
Publication Status
Published
Coverage Spatial
United States
Date Publish Online
2020-04-17