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Cushing syndrome

Last reviewed: 8 Apr 2025
Last updated: 10 Dec 2024

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
Full details

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
  • hirsutism
Full details

Risk factors

  • exogenous corticosteroid use
  • pituitary adenoma
  • adrenal adenoma
  • adrenal carcinoma
  • neuroendocrine tumors
  • thoracic or bronchogenic carcinoma
Full details

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
Full details

Tests to avoid

  • serum cortisol
Full details

Tests to consider

  • plasma dehydroepiandrosterone sulfate (DHEAS) level
  • morning plasma adrenocorticotropic hormone (ACTH)
  • pituitary MRI
  • adrenal CT
  • high-dose dexamethasone suppression test
  • inferior petrosal sinus sampling (IPSS)
  • CT of chest, abdomen, and pelvis
  • MRI chest
  • octreotide scanning
  • gallium-68 DOTATATE PET/CT
Full details

Treatment algorithm

ONGOING

Cushing disease (adrenocorticotropic hormone [ACTH]-secreting pituitary tumor)

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)

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 Pituitary Society's Board of Directors. She holds a research grant to the University for Clinical Studies as Principal Investigator for Recordati and Strongbridge, and is an occasional Scientific Consultant for Recordati, HRA Pharma, and Sparrow. 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, previous contributors to 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

Disclosures

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

Disclosures

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

Disclosures

MAH declares that he has no competing interests.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Sharma ST, Nieman LK, Feelders RA. Cushing's syndrome: epidemiology and developments in disease management. Clin Epidemiol. 2015 Apr 17;7:281-93.Full text  Abstract

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.Full text  Abstract

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.Full text  Abstract

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. Abstract

Ritzel K, Beuschlein F, Mickisch A, et al. Clinical review: outcome of bilateral adrenalectomy in Cushing's syndrome: a systematic review. J Clin Endocrinol Metab. 2013 Oct;98(10):3939-48.Full text  Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
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  • Patient information

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    More Patient information
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