Primary Adrenal Insufficiency Guideline Resources

Full Guideline: Diagnosis and Treatment of Primary Adrenal Insufficiency
JCEM | February 2016

Stefan R. Bornstein (chair), Bruno Allolio, Wiebke Arlt, Andreas Barthel, Andrew Don-Wauchope, Gary D. Hammer, Eystein S. Husebye, Deborah P. Merke, M. Hassan Murad, Constantine A. Stratakis, and David J. Torpy

The 2016 guideline addresses:

  • Treating and diagnosing patients during and after an adrenal crisis
  • Testing and diagnosing in optimal circumstances
  • Setting a regimen for glucocorticoid and mineralocorticoid replacement
  • Treatment and monitoring in pregnancy and childhood
  • Managing adrenal crisis in diagnosed individuals
  • Monitoring and additional screening


Resources



Essential Points

The Endocrine Society recommends that acutely ill patients who have unexplained symptoms undergo diagnostic testing to rule out primary adrenal insufficiency. Those who have severe symptoms of the condition or adrenal crisis should undergo immediate treatment with medication until diagnostic test results are available. Health care providers should conduct a corticotropin stimulation test to confirm the diagnosis when the patient’s condition allows.

  • Patients should undergo a blood test to measure levels of adrenocorticotropic hormone (ACTH) – the hormone that signals the adrenal glands to produce cortisol – to establish a primary adrenal insufficiency diagnosis.
  • As part of the diagnostic process, patients should have blood tests to measure the levels of the hormones renin and aldosterone. This test determines if a person has a deficiency of the hormones used to regulate the balance of salt and water in the body.
  • Patients who have a confirmed diagnosis of primary adrenal insufficiency should undergo glucocorticoid replacement therapy—typically with hydrocortisone (cortisol), the glucocorticoid hormone naturally produced by the adrenal glands.
  • People who have primary adrenal insufficiency and a confirmed aldosterone deficiency should undergo replacement therapy—typically with the synthetic hormone fludrocortisone—to maintain the body’s salt and water balance. Anyone receiving this therapy should be monitored by testing blood electrolyte levels and checking for symptoms like salt craving, light-headedness, blood pressure changes and swelling of the legs and feet.

Summary of Recommendations

+ 1.0 Who should be tested and how?

+ 2.0 Optimal diagnostic tests

+ 3.0 Treatment of primary adrenal insufficiency in adults

+ 4.0 Management and prevention of adrenal crisis in patients with PAI

+ 5.0 Additional monitoring requirement