Experts recommend immediate treatment for severe primary adrenal insufficiency symptoms

The Endocrine Society today issued a Clinical Practice Guideline (CPG) on diagnosis and treatment of primary adrenal insufficiency, a condition commonly known as Addison's disease that occurs when the body produces too little of the hormone cortisol.

The CPG, entitled "Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline," was published online and will appear in the February 2016 print issue of the Journal of Clinical Endocrinology & Metabolism (JCEM), a publication of the Endocrine Society.

Primary adrenal insufficiency is a rare, potentially life-threatening condition that occurs when the adrenal glands located on top of the kidneys do not work properly. The adrenal glands produce cortisol, a hormone essential for the body's response to stress, maintaining blood pressure and cardiovascular function, keeping the immune system in check, and converting fat, carbohydrates and proteins into energy. When an individual develops primary adrenal insufficiency, they may experience symptoms such as weight loss, fatigue, muscle weakness, decreased appetite, nausea, vomiting and diarrhea.

"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, U.K., and chair of the task force that authored the guideline. "Postponing treatment of more severe symptoms raises the risk of death. Severe symptoms need to be treated immediately, even if a test still needs to be conducted to confirm the diagnosis."

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.

Other recommendations from the CPG include:

• 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.