Primary adrenal insufficiency

Last reviewed: 3 Sep 2022
Last updated: 03 May 2022

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • fatigue
  • anorexia
  • weight loss
  • hyperpigmentation
  • acute circulatory collapse with hypotension and tachycardia
  • salt craving
More key diagnostic factors

Other diagnostic factors

  • gastrointestinal symptoms (nausea, vomiting, abdominal pain)
  • postural hypotension
  • arthralgia and myalgia
  • axillary and pubic hair loss in women
Other diagnostic factors

Risk factors

  • female sex
  • adrenocortical autoantibodies
  • adrenal haemorrhage
  • autoimmune diseases
  • coeliac disease
  • tuberculosis (TB)
  • non-TB bacterial infection
  • fungal infection
  • HIV
  • drugs that inhibit cortisol production
  • metastatic malignancy
  • sarcoidosis
More risk factors

Diagnostic investigations

1st investigations to order

  • morning serum cortisol
  • plasma adrenocorticotrophic hormone (ACTH)
  • serum electrolytes
  • urea and creatinine
  • FBC
More 1st investigations to order

Investigations to consider

  • adrenocorticotrophic hormone (ACTH) stimulation test
  • plasma renin activity
  • serum aldosterone
  • serum dehydroepiandrosterone (DHEA) and DHEA sulfate (DHEA-S)
  • adrenal antibodies
  • adrenal CT or MRI
  • insulin hypoglycaemia test
  • overnight single-dose metyrapone test
More investigations to consider

Treatment algorithm

INITIAL

adrenal crisis

ACUTE

minor intercurrent stress

severe intercurrent stress

ONGOING

stable and/or after treatment of acute episode

Contributors

Authors

Laleh Razavi Nematollahi, MD

Assistant Professor of Medicine

Division of Clinical and Molecular Endocrinology and Metabolism

University Hospitals Cleveland Medical Center

Case Western Reserve University

Cleveland

OH

Disclosures

LRN declares that she has no competing interests.

Baha Arafah, MD

Chief, Division of Endocrinology

University Hospitals Cleveland Medical Center

Case Western Reserve University

Cleveland

OH

Disclosures

BA declares that he has no competing interests.

Acknowledgements

Dr Laleh Razavi Nematollahi and Dr Baha Arafah would like to gratefully acknowledge Dr T. Joseph McKenna, Dr Shehzad Basaria, and Dr Milena Braga-Basaria, previous contributors to this topic.

Disclosures

TJM, SB, and MBB declare that they have no competing interests.

Peer reviewers

Rajesh K. Garg, MD

Instructor in Medicine

Brigham and Women's Hospital

Division of Endocrinology

Diabetes and Hypertension

Boston

MA

Disclosures

RKG declares that he has no competing interests.

Antoine Tabarin, MD

Head

Department of Endocrinology

Centre Hospitalier Universitaire de Bordeaux

Pessac

France

Disclosures

AT declares that he has no competing interests.

Blandine Gatta-Cherifi, MD

Department of Endocrinology

Centre Hospitalier Universitaire de Bordeaux

Pessac

France

Disclosures

BGC declares that she has no competing interests.

  • Primary adrenal insufficiency images
  • Differentials

    • Adrenal suppression due to the use of glucocorticoid or other drugs with glucocorticoid activity (e.g., medroxyprogesterone acetate therapy)
    • Central (secondary or tertiary) adrenal insufficiency (pituitary or hypothalamic lesions)
    • Haemochromatosis
    More Differentials
  • Guidelines

    • Guidelines for the management and prevention of acute adrenal insufficiency
    • Adrenal insufficiency - emergency management practice guideline
    More Guidelines
  • Patient leaflets

    Primary adrenal insufficiency (Addison's disease): what is it?

    Primary adrenal insufficiency (Addison's disease): what treatments work?

    More Patient leaflets
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