Primary Adrenal Insufficiency

Over a period of many months, a 58-year-old man with a history of gout and diabetes had episodic muscle cramps, poor appetite, fatigue, and poor glycemic control. A particularly severe episode of cramping necessitated a trip to the hospital because he became pre-syncopal and diaphoretic. On admission, he was noted to be hypotensive and dehydrated. Fasting cortisol levels were consistent with the diagnosis of primary adrenal insufficiency.

Primary adrenal insufficiency, or Addison’s disease, occurs in all age groups and affects both men and women equally. It occurs in about 1 in 100,000 persons. This endocrine disorder usually presents with muscle fatigue, weight loss, hypotension, and sometimes darkening of the skin in both exposed and non-exposed parts of the body (as occurred in this patient).

About 70% of reported cases are caused by autoimmune disorders. Insufficiency occurs when more than 90% of the adrenal cortex has been destroyed. Twenty percent of reported cases are attributable to tuberculosis.

Because the symptoms progress slowly, it often takes a stressful event to precipitate a full crisis. Symptoms of an Addisonian crisis include sudden penetrating pain the low back and abdomen or legs, dehydration, hypotension, and loss of consciousness. The most specific test is the adrenocorticotropic hormone (ACTH) stimulation test.

Treatment consists of cortisol replacement with hydrocortisone tablets and close follow-up.n