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Endocrine Abstracts (2019) 63 P431 | DOI: 10.1530/endoabs.63.P431

1Department of Clinical Science, University of Bergen, Bergen, Norway; 2K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway; 3Department of Medicine, Örebro University Hospital, Örebro, Sweden; 4Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden; 5Department of Medicine, Haukeland University Hospital, Bergen, Norway; 6Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway; 7Department of Endocrinology, Stavanger University Hospital, Stavanger, Norway; 8Department of Medicine, Sørlandet Hospital Kristianand, Kristiansand, Norway; 9Department of Medicine, Sørlandet Hospital Arendal, Arendal, Norway; 10Department of Medicine, Vestfold Hospital Trust, Tønsberg, Norway; 11Department of Medicine, Vestre Viken Hospital, Drammen, Norway; 12Department of Endocrinology, Oslo University Hospital, Oslo, Norway; 13Division of Medicine, Akershus University Hospital, Lørenskog, Norway; 14Division of Endocrinology, Innlandet Hospital Trust, Hamar, Norway; 15Division of Internal Medicine, University Hospital of North Norway, Tromsø, Norway; 16Tromsø Endocrine Research Group, Department of Clinical Medicine, UiT the Arctic University of Norway, Tromsø, Norway; 17Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden; 18Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden; 19Department of Endocrinology, Department of Medical and Health Sciences, Department of Clinical and Experimental Medicine, Linköping University, Linköping, Norway; 20Department of Medical Sciences, Uppsala University, Uppsala, Sweden; 21Endocrinology in Charlottenburg, Berlin, Germany.

Background: Current dogma states that in autoimmune Addison’s disease (AAD), all adrenocortical function eventually is lost. Yet growing evidence suggests that a subgroup of patients retain some self-production.

Aim: To explore whether residual production of adrenocortical steroids is present in a subgroup of AAD patients.

Material and methods: In an open non-randomized cross-sectional study, an interim of 22 AAD patients delivered a medication-fasting morning blood sample for analysis of adrenocortical steroids by the highly sensitive and specific liquid chromatography tandem mass spectrometry (LC-MS/MS) method. Before sampling, patients abstained from cortisone acetate or hydrocortisone and fludrocortisone for at least 18 and 24 hours, respectively. Residual function was defined as morning serum cortisol (s-cortisol) and/ and or aldosterone (s-aldosterone) above their corresponding lower limit of quantification (LLOQ). Dehydroepiandrosterone (DHEA) and corticosteroid precursors were also measured.

Results: Twenty-two patients (8 males, 14 females) aged 49.7 (±14.3) years with verified autoimmune adrenal insufficiency were included. Mean disease duration was 18.1 (±13.9) years. Twelve of 22 (54%) patients had morning s-cortisol exceeding the LLOQ of 0.91 nmol/l. Median s-cortisol was low (3.19 nmol/l [0.92–339.6]), but three patients presented with s-cortisol 71, 204 and 339 nmol/l. They had disease durations of 44, 5, and 5 years, respectively. There was no significant correlation between measurable s-cortisol and age (P<0.899), sex (P<0.254) or disease duration (P<0.350). S-aldosterone surpassed the LLOQ of 2.3 pmol/l in three patients (3, 14 and 217 pmol/l, respectively) in which all had detectable s-cortisol as well. Seven patients (32%) had measurable levels of DHEA (median 0.86 nmol/l [0.65–1.65]). Corticosteroid precursors were detected in 5 patients (23%) for 11-deoxycorticosterone (median 0.11 nmol/l [0.03–0.92]), 5 (23%) for 18-hydroxycorticosterone (median 0.25 nmol/l [0.07–3.40]), 5 (23%) for corticosterone (median 4.00 nmol/l [0.17–50.84]), and 4 (18%) for 11-deoxycortisol (median 1.96 nmol/l [0-18–2.15]). All precursors were present in the three patients with the highest fasting s-cortisol. In seven patients (33%) with measurable s-cortisol less than 3.5 nmol/L, none of the precursors were detected.

Conclusion: More than half of the patients had detectable levels of adrenal steroid hormones even years after the diagnosis; three had clinically significant levels of cortisol as well as detectable precursors. Patients with preserved steroid producing-capacity could be candidates for therapy aimed at regenerating and restoring adrenocortical function.

Volume 63

21st European Congress of Endocrinology

Lyon, France
18 May 2019 - 21 May 2019

European Society of Endocrinology 

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