Residual Corticosteroid Production in Autoimmune Addison Disease

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Residual Corticosteroid Production in Autoimmune Addison Disease

Sævik, Åse Bjorvatn (author): Department of Clinical Science, University of Bergen, Norway; K.G. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway,Univ Bergen, Norway

Åkerman, Anna-Karin (author): Karolinska Institutet

Methlie, Paal (author): 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,Univ Bergen, Norway; Haukeland Hosp, Norway

Quinkler, Marcus (author): Endocrinology in Charlottenburg, Berlin, Germany,Endocrinol Charlottenburg, Germany

Jørgensen, Anders Palmstrøm (author): Department of Endocrinology, Oslo University Hospital, Oslo, Norway,Oslo Univ Hosp, Norway

Höybye, Charlotte (author): Karolinska Institutet

Debowska, Aleksandra J. (author): Department of Medicine, Vestfold Hospital Trust, Tønsberg, Norway,Vestfold Hosp Trust, Norway

Nedrebø, Bjørn Gunnar (author): Department of Clinical Science, University of Bergen, Norway; Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway,Univ Bergen, Norway; Haugesund Hosp, Norway

Dahle, Anne Lise (author): Department of Internal Medicine, Haugesund Hospital, Haugesund, Norway,Haugesund Hosp, Norway

Carlsen, Siri (author): Department of Endocrinology, Stavanger University Hospital, Stavanger, Norway,Stavanger Univ Hosp, Norway

Tomkowicz, Aneta (author): Department of Medicine, Sørlandet Hospital, Kristiansand, Norway,Sorlandet Hosp, Norway

Sollid, Stina Therese (author): Department of Medicine, Drammen Hospital, Vestre Viken Health Trust, Drammen, Norway

Nermoen, Ingrid (author): Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway,Akershus Univ Hosp, Norway

Grønning, Kaja (author): Department of Endocrinology, Akershus University Hospital, Lørenskog, Norway,Akershus Univ Hosp, Norway

Dahlqvist, Per (author): Umeå universitet,Avdelningen för medicin,Umea Univ, Sweden

Grimnes, Guri (author): 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,Univ Hosp North Norway, Norway; UiT Arctic Univ Norway, Norway

Skov, Jakob (author): Karolinska Institutet

Finnes, Trine (author): Section of Endocrinology, Innlandet Hospital Trust, Hamar, Norway,Innlandet Hosp Trust, Norway

Valland, Susanna F. (author): Section of Endocrinology, Innlandet Hospital Trust, Hamar, Norway,Innlandet Hosp Trust, Norway

Wahlberg, Jeanette, 1969- (author): Linköpings universitet,Avdelningen för diagnostik och specialistmedicin,Medicinska fakulteten,Region Östergötland, Endokrinmedicinska kliniken

Holte, Synnøve Emblem (author): Department of Medicine, Sørlandet Hospital, Arendal, Norway,Sorlandet Hosp, Norway

Simunkova, Katerina (author): Department of Clinical Science, University of Bergen, Norway,Univ Bergen, Norway

Kämpe, Olle (author): Karolinska Institutet

Husebye, Eystein Sverre (author): 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,Univ Bergen, Norway; Haukeland Hosp, Norway; Karolinska Inst, Sweden

Bensing, Sophie (author): Karolinska Institutet

øksnes, Marianne (author): 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,Univ Bergen, Norway; Haukeland Hosp, Norway; Karolinska Inst, Sweden

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Department of Clinical Science, University of Bergen, Norway; KG. Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway Univ Bergen, Norway (creator_code:org_t)

2020-05-11
2020
English.
In: Journal of Clinical Endocrinology and Metabolism. - Washington : Oxford University Press. - 0021-972X .- 1945-7197. ; 105:7, s. 2430-2441

Related links:: https://doi.org/10.1...; show more...; https://academic.oup...; https://umu.diva-por... (primary) (Raw object); https://liu.diva-por... (primary) (Raw object); https://urn.kb.se/re...; https://doi.org/10.1...; https://urn.kb.se/re...; https://urn.kb.se/re...; http://kipublication...; show less...

Journal article (peer-reviewed)

Abstract Subject headings

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.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Endokrinologi och diabetes (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Endocrinology and Diabetes (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Reumatologi och inflammation (hsv//swe)
MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Rheumatology and Autoimmunity (hsv//eng)

Keyword

Adrenal failure
autoimmune Addison disease
adrenal steroids
cortisol
primary adrenal insufficiency
residual function

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ref (subject category)
art (subject category)

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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Endocrinology an ...

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