Last reviewed: 1 Nov 2021
Last updated: 17 Nov 2020

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • facial plethora
  • supraclavicular fullness
  • violaceous striae
  • absence of pregnancy
  • menstrual irregularities
  • absence of malnutrition
  • absence of alcoholism
  • absence of physiological 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
  • hirsutism

Risk factors

  • exogenous corticosteroid use
  • pituitary adenoma
  • adrenal adenoma
  • adrenal carcinoma
  • neuroendocrine tumours
  • thoracic or bronchogenic carcinoma

Diagnostic investigations

1st investigations 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

Investigations to consider

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

Treatment algorithm

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 the Pituitary Society's President and sits on its Board of Directors. She holds a research grant to the University for Clinical Studies as Principal Investigator for Novartis, Millendo, and Strongbridge, and is an occasional Scientific Consultant for Novartis and Strongbridge. 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.

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