Primary adrenocortical insufficiency

Primary Adrenocortical Insufficiency

Primary adrenocortical insufficiency, also known as Addison’s disease, is an endocrine disorder in which the adrenal glands do not produce enough of the hormone cortisol. This rare disorder affects approximately 1 in 100,000 people, and is more common among women than men.

Symptoms of primary adrenocortical insufficiency include:

  • Fatigue
  • Weight loss and decreased appetite
  • Muscle weakness and joint pain
  • Abdominal pain
  • Low blood pressure
  • Hyperpigmentation
  • Salt cravings

When left untreated, primary adrenocortical insufficiency can lead to more serious health issues, including Addisonian crisis, a life-threatening episode characterized by severe dehydration, low blood pressure, extreme exhaustion, and confusion.

Primary adrenocortical insufficiency is caused by the destruction of the adrenal cortex. In some cases, this destruction may result from an autoimmune disorder, in which the immune system mistakenly attacks the adrenal glands. Other causes include infections such as tuberculosis, and certain tumors.

Diagnosis is usually made based on patient history, followed by a blood test; an ACTH stimulation test can also be used to measure cortisol levels. Treatment typically involves hormone replacement therapy, with the patient taking cortisol and aldosterone supplements daily. It is important to adhere to this treatment in order to manage symptoms and avoid an Addisonian crisis.