DISEASES OF THE ADRENAL CORTEX

PRIMARY ADRENAL INSUFFICIENCY (Addison Disease)

EPIDEMIOLOGY:
Addison disease is an uncommon disorder with a prevalence of about 90-140 cases per million and an
annual incidence of about 5-6 cases per million in the United States.

ETIOLOGY:
 Autoimmunity,
 Bilateral adrenal infiltrative disease,
 Bilateral adrenal hemorrhage may occur with sepsis,
 Adrenoleukodystrophy is an X-linked peroxisomal disorder causing accumulation of very long-
chain fatty acids in the adrenal cortex, testes, brain, and spinal cord,
 Congenital adrenal insufficiency.

ESSENTIALS OF DIAGNOSIS
 Deficiency of cortisol and mineralocorticoid from destruction of the adrenal cortex.
 Weakness, vomiting, diarrhea; abdominal pain, arthralgias; amenorrhea.
 Increased skin pigmentation, especially of creases, pressure areas, and nipples.
 Hypovolemic hypotension, small heart.
 Hyponatremia; hyperkalemia (may be absent with vomiting and diarrhea); hypoglycemia;
eosinophilia. Elevated plasma ACTH level; cosyntropin unable to stimulate serum cortisol to 20
mcg/dL (550 nmol/L) or more.
 Acute adrenal crisis: above manifestations become critical, with fever, shock, confusion, coma,
death.
 General Considerations Primary adrenal insufficiency (Addison disease) is caused by dysfunction
or absence of the adrenal cortices. It is distinct from secondary adrenal insufficiency caused by
deficient secretion of ACTH.

PATHOPHYSIOLOGY:
Addison's disease occurs when the adrenal glands do not produce enough cortisol and, in some
cases, aldosterone. Adrenal insufficiency may arise due to insufficient release of cortisol from the adrenal
glands. Insufficient cortisol secretion may be due to adrenal dysgenesis (the gland does not form adequately
during development), impaired steroidogenesis (the gland is present but is biochemically unable to
produce cortisol) or adrenal destruction (disease processes leading to the gland being damaged).

SIGN AND SYMPTOMS:

Over 90% of patients complain of fatigue, reduced stamina, weakness, anorexia, and weight loss. Over
80% of affected patients present with symptoms of orthostatic hypotension (aggravated by dehydration
caused by nausea or vomiting), lightheadedness with standing, salt craving, and eventually
hyperpigmentation of skin and gums.
TREATMENT:
Corticosteroids and fludrocortisone must be prescribed in liberal amounts with automatic refills to avoid
the patient’s running out of medication. It is also advisable to prescribe a routine antiemetic such as
ondansetron ODT 8-mg tablets to be taken every 8 hours for nausea. Parenteral hydrocortisone (Solu-
Cortef) 100 mg is also prescribed for patient self-injection in the event of vomiting. Patients must receive
advance instructions to seek medical attention at an emergency facility immediately in the event of
vomiting or severe illness. All infections should be treated immediately and vigorously, with
hydrocortisone administered at appropriately increased doses.