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Autoimmune primary adrenal insufficiency

MedGen UID:
543526
Concept ID:
C0271737
Disease or Syndrome
Synonym: Addison's disease due to autoimmunity
SNOMED CT: Autoimmune Addison's disease (76715008); Addison's disease due to autoimmunity (76715008); Autoimmune adrenal atrophy (76715008); Autoimmune adrenalitis (76715008); Addison disease due to autoimmunity (76715008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0100480
Orphanet: ORPHA85138

Definition

A chronic and rare endocrine disorder due to autoimmune destruction of the adrenal cortex and resulting in a glucocorticoid and mineralocorticoid deficiency. Properly speaking, it designates autoimmune adrenalitis, but it is a term commonly used to describe any form of chronic primary adrenal insufficiency (CPAI). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutoimmune primary adrenal insufficiency

Professional guidelines

PubMed

Mourinho Bala N, Gonçalves RS, Serra Caetano J, Cardoso R, Dinis I, Mirante A
J Clin Res Pediatr Endocrinol 2022 Aug 25;14(3):308-312. Epub 2022 May 31 doi: 10.4274/jcrpe.galenos.2022.2021-11-9. PMID: 35633647Free PMC Article
Kamrath C, Hartmann MF, Wudy SA
Steroids 2019 Oct;150:108426. Epub 2019 Jun 20 doi: 10.1016/j.steroids.2019.108426. PMID: 31228484

Recent clinical studies

Therapy

Brabham C, Cohen S, Covés-Datson E, Carbo AR
Am J Med 2023 Dec;136(12):e237-e238. Epub 2023 Sep 10 doi: 10.1016/j.amjmed.2023.08.018. PMID: 37699498
Kamrath C, Hartmann MF, Wudy SA
Steroids 2019 Oct;150:108426. Epub 2019 Jun 20 doi: 10.1016/j.steroids.2019.108426. PMID: 31228484
Erichsen MM, Løvås K, Skinningsrud B, Wolff AB, Undlien DE, Svartberg J, Fougner KJ, Berg TJ, Bollerslev J, Mella B, Carlson JA, Erlich H, Husebye ES
J Clin Endocrinol Metab 2009 Dec;94(12):4882-90. Epub 2009 Oct 26 doi: 10.1210/jc.2009-1368. PMID: 19858318

Prognosis

Bratland E, Skinningsrud B, Undlien DE, Mozes E, Husebye ES
J Clin Endocrinol Metab 2009 Dec;94(12):5117-24. Epub 2009 Nov 4 doi: 10.1210/jc.2009-1115. PMID: 19890026
Erichsen MM, Løvås K, Skinningsrud B, Wolff AB, Undlien DE, Svartberg J, Fougner KJ, Berg TJ, Bollerslev J, Mella B, Carlson JA, Erlich H, Husebye ES
J Clin Endocrinol Metab 2009 Dec;94(12):4882-90. Epub 2009 Oct 26 doi: 10.1210/jc.2009-1368. PMID: 19858318

Clinical prediction guides

Zdrojowy-Wełna A, Halupczok-Żyła J, Słoka N, Syrycka J, Gojny Ł, Bolanowski M
Front Endocrinol (Lausanne) 2022;13:996157. Epub 2022 Nov 2 doi: 10.3389/fendo.2022.996157. PMID: 36407318Free PMC Article
Mourinho Bala N, Gonçalves RS, Serra Caetano J, Cardoso R, Dinis I, Mirante A
J Clin Res Pediatr Endocrinol 2022 Aug 25;14(3):308-312. Epub 2022 May 31 doi: 10.4274/jcrpe.galenos.2022.2021-11-9. PMID: 35633647Free PMC Article
Kamrath C, Hartmann MF, Wudy SA
Steroids 2019 Oct;150:108426. Epub 2019 Jun 20 doi: 10.1016/j.steroids.2019.108426. PMID: 31228484
Bratland E, Skinningsrud B, Undlien DE, Mozes E, Husebye ES
J Clin Endocrinol Metab 2009 Dec;94(12):5117-24. Epub 2009 Nov 4 doi: 10.1210/jc.2009-1115. PMID: 19890026
Erichsen MM, Løvås K, Skinningsrud B, Wolff AB, Undlien DE, Svartberg J, Fougner KJ, Berg TJ, Bollerslev J, Mella B, Carlson JA, Erlich H, Husebye ES
J Clin Endocrinol Metab 2009 Dec;94(12):4882-90. Epub 2009 Oct 26 doi: 10.1210/jc.2009-1368. PMID: 19858318

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