People with primary adrenal insufficiency symptoms should be diagnosed and treated immediately, new guideline says

By John Murphy, MDLinx
Published January 14, 2016

Key Takeaways

Acutely ill patients who have unexplained symptoms or signs that might indicate primary adrenal insufficiency (PAI) should be treated immediately, and not wait for diagnostic test results, according to a new clinical practice guideline issued online January 13, 2016 by the Endocrine Society.

The new practice guideline also recommends diagnostic testing for all patients with clinical symptoms or signs of PAI (also referred to as Addison’s disease), with a particular low diagnostic and therapeutic threshold for acutely ill patients.

“Diagnosing primary adrenal insufficiency remains challenging because many of the symptoms are associated with a variety of health conditions,” said Stefan R. Bornstein, MD, PhD, of the Universitätsklinikum in Dresden, Germany, and King’s College in London, UK, and chair of the task force that authored the guideline.

Except for the characteristic craving for salt, symptoms of PAI are nonspecific, such as weakness, fatigue, musculoskeletal pain, weight loss, abdominal pain, depression, and anxiety. As a result, the diagnosis is often delayed, which can result in an acute life-threatening adrenal crisis.

In any patient with such symptoms or signs, health care providers should conduct a short corticotropin test (250 μg), which is the “gold standard” diagnostic tool to establish the diagnosis of PAI, the guideline states.

Symptoms and signs of severe PAI may include weight loss, hypotension, hyponatremia, hyperkalemia, fever, abdominal pain, hyperpigmentation, and—especially in children—hypoglycemia. Patients who present with severe symptoms or who are in adrenal crisis should be treated right away.

PAI occurs when the adrenal cortex doesn’t produce sufficient amounts of glucocorticoids and/or mineralocorticoids. It’s a severe and potentially life-threatening condition due to the central role of these hormones in energy, salt, and fluid homeostasis, according to the Endocrine Society.

“Postponing treatment of more severe symptoms raises the risk of death,” Dr. Bornstein added. “Severe symptoms need to be treated immediately, even if a test still needs to be conducted to confirm the diagnosis.”

Patients with suspected adrenal crisis should be treated immediately with a parenteral injection of hydrocortisone (100 mg; 50 mg/m2 for children), followed by appropriate fluid resuscitation and additional hydrocortisone dosing (continuous IV or recurrent injection), the guideline recommends.

Other recommendations include:

  • Patients should undergo a blood test to measure levels of adrenocorticotropic hormone (ACTH) to establish PAI diagnosis.
  • Patients should also have blood tests performed to measure levels of the hormones renin and aldosterone to identify mineralocorticoid deficiency.
  • Patients who have a confirmed diagnosis of PAI should undergo glucocorticoid replacement therapy, typically with hydrocortisone.
  • Patients with PAI and a confirmed aldosterone deficiency should undergo replacement therapy, typically with 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.
  • Patients should be educated about stress dosing and equipped with a steroid card and glucocorticoid preparation for parenteral emergency administration.

The clinical practice guideline, titled “Diagnosis and Treatment of Primary Adrenal Insufficiency,” will also appear in the February 2016 print issue of the Journal of Clinical Endocrinology & Metabolism. It is now available online from the Endocrine Society.

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