Cushing syndrome
Summary
Definition
History and exam
Key diagnostic factors
- facial plethora
- supraclavicular fullness
- violaceous striae
- absence of pregnancy
- menstrual irregularities
- absence of malnutrition
- absence of alcoholism
- absence of physiologic stress
- linear growth deceleration in children
Other diagnostic factors
- female sex
- hypertension
- glucose intolerance or diabetes mellitus
- premature osteoporosis or unexplained fractures
- weight gain and central obesity
- acne
- psychiatric symptoms
- decreased libido
- easy bruisability
- weakness
- facial rounding
- dorsocervical fat pads
- unexplained nephrolithiasis
- venothrombolic event
- virilization
Risk factors
- exogenous corticosteroid use
- pituitary adenoma
- adrenal adenoma
- adrenal carcinoma
- neuroendocrine tumors
- thoracic or bronchogenic carcinoma
Diagnostic tests
1st tests to order
- urine pregnancy test
- serum glucose
- late-night salivary cortisol
- 1 mg overnight dexamethasone suppression test
- 24-hour urinary free cortisol
- 48-hour 2 mg (low-dose) dexamethasone suppression test
Tests to avoid
- serum cortisol
Tests to consider
- morning plasma adrenocorticotropic hormone (ACTH)
- plasma dehydroepiandrosterone sulfate (DHEAS) level
- pituitary MRI
- adrenal imaging
- high-dose dexamethasone suppression test
- inferior petrosal sinus sampling (IPSS)
- CT of chest, abdomen, and pelvis
- MRI chest, abdomen, and pelvis
- fluorodeoxyglucose positron emission tomography (FDG-PET) or FDG-PET/CT
- octreotide scanning
- gallium-68 dotatate PET/CT
Treatment algorithm
Cushing disease (adrenocorticotropic hormone [ACTH]-secreting pituitary adenoma)
ectopic ACTH or corticotropin-releasing hormone (CRH) syndrome
ACTH-independent due to unilateral adrenal carcinoma or adenoma
ACTH-independent due to bilateral adrenal disease (hyperplasia or adenoma)
mild autonomous cortisol secretion (previously known as subclinical Cushing syndrome)
Contributors
Authors
Maria Fleseriu, MD, FACE
Professor of Medicine (Endocrinology) and Neurological Surgery
Director
Pituitary Center
Oregon Health & Science University
Portland
OR
Disclosures
MF is on the Endocrine Society's Board of Directors. She holds a research grant to the university for clinical studies as principal investigator for Crinetics and Sparrow Pharmaceuticals, and is an occasional scientific consultant for Crinetics, Recordati, Sparrow Pharmaceuticals, and Xeris Pharmaceuticals. MF is an author of several references cited in this topic.
Acknowledgements
Dr Maria Fleseriu would like to gratefully acknowledge Dr Ty Carroll and Dr James Findling, contributors to a previous version of this topic.
Disclosures
TC is an author of a number of references cited in this topic. He is an investigator in clinical trials sponsored by Corcept. JF is an author of a number of references cited in this topic. He is a consultant for, and investigator in, clinical trials sponsored by Corcept and Novartis.
Peer reviewers
Paul M. Stewart, FRCP FMedSci
Professor of Medicine
Director of Research
College of Medical and Dental Sciences
University of Birmingham
Honorary Consultant Physician
Queen Elizabeth Hospital
Birmingham
UK
Disclosures
PMS declares that he has no competing interests.
Antoine Tabarin, MD
Head
Department of Endocrinology
University Hospital of Bordeaux
Pessac
France
Disclosures
AT declares that he has no competing interests.
Liliana Contrersas, MD
Chief
Endocrine Research Department
Instituto de Investigaciones Médicas A. Lanari
University of Buenos Aires and IDIM-CONICET
Buenos Aires
Argentina
Declarações
LC declares that she has no competing interests.
Philip R. Orlander, MD
Professor of Medicine
Director
Division of Endocrinology, Diabetes & Metabolism
University of Texas Medical School
Houston
TX
Declarações
PRO declares that he has no competing interests.
Mouhammed Amir Habra, MD, FACP, FACE
Assistant Professor
Department of Endocrine Neoplasia and Hormonal Disorders
Division of Internal Medicine
University of Texas MD Anderson Cancer Center
Houston
TX
Declarações
MAH declares that he has no competing interests.
Créditos aos pareceristas
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Declarações
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Referências
Principais artigos
Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75.Texto completo Resumo
Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing’s syndrome: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2015 Aug;100(8):2807-31.Texto completo Resumo
Feelders RA, Newell-Price J, Pivonello R, et al. Advances in the medical treatment of Cushing's syndrome. Lancet Diabetes Endocrinol. 2019 Apr;7(4):300-12. Resumo
Artigos de referência
Uma lista completa das fontes referenciadas neste tópico está disponível para os usuários com acesso total ao BMJ Best Practice.
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