Experts Recommend Immediate Treatment for Severe Primary Adrenal Insufficiency Symptoms

January 13, 2016

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Endocrine Society publishes Clinical Practice Guideline on condition known as Addison’s disease

Washington, DC - 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.

The Hormone Health Network offers resources on adrenal insufficiency at http://www.hormone.org/questions-and-answers/2010/adrenal-insufficiency.

Other members of the Endocrine Society task force that developed this CPG include: Bruno Allolio, of University of Würzburg in Würzburg, Germany; Wiebke Arlt of the University of Birmingham in Birmingham, U.K.; Andreas Barthel of the Universitätsklinikum Dresden and Endokrinologikum Ruhr in Bochum, Germany; Andrew Don-Wauchope of McMaster University and Hamilton Regional Laboratory Medicine Program in Hamilton, Ontario, Canada; Gary D. Hammer of the University of Michigan in Ann Arbor, Michigan; Eystein S. Husebye of the University of Bergen and Haukeland University Hospital in Bergen, Norway; Deborah P. Merke of the National Institutes of Health Clinical Center in Bethesda, MD; M. Hassan Murad of the Mayo Clinic in Rochester, MN; Constantine A. Stratakis of the National Institutes of Health’s Eunice Kennedy Shriver National Institute of Child Health and Human Development in Bethesda, MD; and David J. Torpy of the Royal Adelaide Hospital, University of Adelaide in Adelaide, Australia.

The Society established the CPG Program to provide endocrinologists and other clinicians with evidence-based recommendations in the diagnosis and treatment of endocrine-related conditions. Each CPG is created by a task force of topic-related experts in the field. Task forces rely on evidence-based reviews of the literature in the development of CPG recommendations. The Endocrine Society does not solicit or accept corporate support for its CPGs. All CPGs are supported entirely by Society funds.

The CPG was co-sponsored by the European Society of Endocrinology and the American Association for Clinical Chemistry.

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