Dissemin is shutting down on January 1st, 2025

Published in

De Gruyter, Clinical Chemistry and Laboratory Medicine, 6(56), p. 896-900, 2018

DOI: 10.1515/cclm-2017-1108

Links

Tools

Export citation

Search in Google Scholar

The natural history of autoimmune Addison’s disease with a non-classical presentation: a case report and review of literature

Distributing this paper is prohibited by the publisher
Distributing this paper is prohibited by the publisher

Full text: Unavailable

Red circle
Preprint: archiving forbidden
Red circle
Postprint: archiving forbidden
Orange circle
Published version: archiving restricted
Data provided by SHERPA/RoMEO

Abstract

Abstract Autoimmune Addison’s disease (AAD) is the most frequent cause of adrenocortical insufficiency. The natural history of AAD usually comprises five consecutive stages with the first stage characterized by the increase of plasma renin consistent with the impairment of pars glomerulosa, which is usually the first affected layer of the adrenal cortex. We describe a 19-year-old female with Hashimoto’s thyroiditis (HT) who underwent an autoantibody screening due to having the personal and family history of other autoimmune diseases in the absence of relevant clinical manifestations. She was positive for adrenal cortex autoantibodies (ACA) and steroid 21-hydroxylase autoantibodies (21-OH Ab) at high titers. She had increased basal levels of ACTH with normal basal cortisol not responding to ACTH stimulation, reduced levels of dehydroepiandrosterone-sulfate but normal levels of orthostatic renin and aldosterone. This scenario was consistent with a subclinical AAD presenting with first impairments in pars fasciculata and reticularis and conserved pars glomerulosa function. Only subsequently, progressive deficiency in pars glomerulosa function has become evident. Review of the literature showed that there was only one case, reported to date, with a similar atypical natural history of AAD. The strategies for screening for ACA/21-OH Ab in patients with HT are discussed.