Primary adrenal insufficiency, or
Addison disease, has many causes, the most common of which is autoimmune adrenalitis. Autoimmune adrenalitis results from destruction of the
adrenal cortex, which leads to deficiencies in
glucocorticoids,
mineralocorticoids, and adrenal
androgens. In the
United States and
Western Europe, the estimated
prevalence of
Addison disease is one in 20,000
persons; therefore, a high clinical suspicion is needed to avoid misdiagnosing a
life-threatening adrenal crisis (i.e.,
shock,
hypotension, and volume depletion). The clinical manifestations before an adrenal crisis are subtle and can include
hyperpigmentation,
fatigue,
anorexia,
orthostasis,
nausea,
muscle and
joint pain, and
salt craving.
Cortisol levels decrease and
adrenocorticotropic hormone levels increase. When clinically suspected,
patients should undergo a
cosyntropin stimulation test to confirm the
diagnosis.
Treatment of
primary adrenal insufficiency requires replacement of
mineralocorticoids and
glucocorticoids. During times of stress (e.g., illness, invasive
surgical procedures), stress-
dose glucocorticoids are required because destruction of the
adrenal glands prevents an adequate physiologic response. Management of
primary adrenal insufficiency or autoimmune adrenalitis requires vigilance for concomitant
autoimmune diseases; up to 50% of
patients develop another autoimmune disorder during their lifetime.