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Primary adrenal insufficiency

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of primary adrenal insufficiency are prepared by our editorial team based on guidelines from the Endocrine Society (ES 2016). ...
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Screening and diagnosis

Indications for testing: as per ES 2016 guidelines, obtain diagnostic testing to exclude PAI in acutely ill patients with otherwise unexplained symptoms or signs suggestive of PAI (volume depletion, hypotension, hyponatremia, hyperkalemia, fever, abdominal pain, hyperpigmentation or, especially in children, hypoglycemia).
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Diagnostic investigations

Corticotropin stimulation test: as per ES 2016 guidelines, perform a corticotropin stimulation test in patients with clinical symptoms or signs suggesting PAI, when the patient's condition and circumstance allow.
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  • Morning cortisol

  • Plasma ACTH

  • Plasma renin and aldosterone

  • Evaluation for underlying etiology

Medical management

Corticosteroid replacement: as per ES 2016 guidelines, administer corticosteroid therapy to all patients with confirmed PAI.
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  • Mineralocorticoid replacement

  • DHEA replacement

  • Management of adrenal crisis

Nonpharmacologic interventions

Salt intake: as per ES 2016 guidelines, advise all patients with confirmed aldosterone deficiency not to restrict their salt intake.
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Specific circumstances

Pregnant patients: as per ES 2016 guidelines, consider using hydrocortisone over cortisone acetate, prednisolone, or prednisone, in pregnant women with PAI.
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  • Pediatric patients

Patient education

Genetic counseling: as per ES 2016 guidelines, consider genetic counseling for patients with PAI due to monogenic disorders.
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  • Stress dose corticosteroids

  • Corticosteroid injection kits

Preventative measures

Prevention of adrenal crisis: as per ES 2016 guidelines, consider adjusting corticosteroid dose according to severity of illness or magnitude of the stressor to prevent adrenal crisis.
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Follow-up and surveillance

Serial clinical assessment: as per ES 2016 guidelines, consider referring adults and children with PAI to be seen by an endocrinologist or a healthcare provider with endocrine expertise at least annually. Infants should be seen at least every 3 to 4 months.
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  • Monitoring of corticosteroid replacement

  • Monitoring of mineralocorticoid replacement

  • Monitoring of DHEA replacement

  • Periodic screening for autoimmune diseases

Quality improvement

Medical alert bracelet: as per ES 2016 guidelines, equip all patients with a steroid emergency card and medical alert identification to alert health personnel regarding the need for increased corticosteroid doses in order to avert or treat adrenal crisis, and regarding the need for immediate parenteral steroid treatment in the event of an emergency.