Residual Corticosteroid Production in Autoimmune Addison Disease.

Autor:	Sævik ÅB; Department of Clinical Science, University of Bergen, Norway.; K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway., Åkerman AK; Department of Medicine, Örebro University Hospital, Örebro, Sweden.; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden., Methlie P; Department of Clinical Science, University of Bergen, Norway.; K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway.; Department of Medicine, Haukeland University Hospital, Bergen, Norway., Quinkler M; Endocrinology in Charlottenburg, Berlin, Germany., Jørgensen AP; Department of Endocrinology, Oslo University Hospital, Oslo, Norway., Höybye C; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.; Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden., Debowska AJ; Department of Medicine, Vestfold Hospital Trust, Tønsberg, Norway., Nedrebø BG; Department of Clinical Science, University of Bergen, Norway.; Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway., Dahle AL; Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway., Carlsen S; Department of Endocrinology, Stavanger University Hospital, Stavanger, Norway., Tomkowicz A; Department of Medicine, Sørlandet Hospital, Kristiansand, Norway., Sollid ST; Department of Medicine, Drammen Hospital, Vestre Viken Health Trust, Drammen, Norway., Nermoen I; Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway., Grønning K; Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway., Dahlqvist P; Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden., Grimnes G; Division of Internal Medicine, University Hospital of North Norway, Tromsø, Norway.; Tromsø Endocrine Research Group, Department of Clinical Medicine, UiT the Arctic University of Norway, Tromsø, Norway., Skov J; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden., Finnes T; Section of Endocrinology, Innlandet Hospital Trust, Hamar, Norway., Valland SF; Section of Endocrinology, Innlandet Hospital Trust, Hamar, Norway., Wahlberg J; Department of Endocrinology and Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden., Holte SE; Department of Medicine, Sørlandet Hospital, Arendal, Norway., Simunkova K; Department of Clinical Science, University of Bergen, Norway., Kämpe O; K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway.; Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden.; Department of Medicine (Solna), Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden., Husebye ES; Department of Clinical Science, University of Bergen, Norway.; K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway.; Department of Medicine, Haukeland University Hospital, Bergen, Norway.; Department of Medicine (Solna), Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden., Bensing S; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.; Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden., Øksnes M; Department of Clinical Science, University of Bergen, Norway.; K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway.; Department of Medicine, Haukeland University Hospital, Bergen, Norway.; Department of Medicine (Solna), Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden.
Jazyk:	angličtina
Zdroj:	The Journal of clinical endocrinology and metabolism [J Clin Endocrinol Metab] 2020 Jul 01; Vol. 105 (7).
DOI:	10.1210/clinem/dgaa256
Abstrakt:	Context: Contrary to current dogma, growing evidence suggests that some patients with autoimmune Addison disease (AAD) produce corticosteroids even years after diagnosis. Objective: To determine frequencies and clinical features of residual corticosteroid production in patients with AAD. Design: Two-staged, cross-sectional clinical study in 17 centers (Norway, Sweden, and Germany). Residual glucocorticoid (GC) production was defined as quantifiable serum cortisol and 11-deoxycortisol and residual mineralocorticoid (MC) production as quantifiable serum aldosterone and corticosterone after > 18 hours of medication fasting. Corticosteroids were analyzed by liquid chromatography-tandem mass spectrometry. Clinical variables included frequency of adrenal crises and quality of life. Peak cortisol response was evaluated by a standard 250 µg cosyntropin test. Results: Fifty-eight (30.2%) of 192 patients had residual GC production, more common in men (n = 33; P < 0.002) and in shorter disease duration (median 6 [0-44] vs 13 [0-53] years; P < 0.001). Residual MC production was found in 26 (13.5%) patients and associated with shorter disease duration (median 5.5 [0.5-26.0] vs 13 [0-53] years; P < 0.004), lower fludrocortisone replacement dosage (median 0.075 [0.050-0.120] vs 0.100 [0.028-0.300] mg; P < 0.005), and higher plasma renin concentration (median 179 [22-915] vs 47.5 [0.6-658.0] mU/L; P < 0.001). There was no significant association between residual production and frequency of adrenal crises or quality of life. None had a normal cosyntropin response, but peak cortisol strongly correlated with unstimulated cortisol (r = 0.989; P < 0.001) and plasma adrenocorticotropic hormone (ACTH; r = -0.487; P < 0.001). Conclusion: In established AAD, one-third of the patients still produce GCs even decades after diagnosis. Residual production is more common in men and in patients with shorter disease duration but is not associated with adrenal crises or quality of life. (© Endocrine Society 2020.)
Databáze:	MEDLINE
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