3
Diagnosis
Table 1. Clinical Features of Adrenal Insufficiency and
Adrenal Crisis
Symptom Signs
Routine Laboratory
Tests
Adrenal Insufficiency
Fatigue Hyperpigmentation (primary
only), particularly of sun-exposed
areas, skin creases, mucosal
membranes, scars, areola of breast
Hyponatremia
Weight loss Low blood pressure with
increased postural drop
Hyperkalemia
Postural dizziness Failure to thrive in children Uncommon: hypoglycemia,
hypercalcemia
Anorexia, abdominal
discomfort
Adrenal crisis
Severe weakness Hyponatremia
Syncope Hypotension Hyperkalemia
Abdominal pain, nausea,
vomiting ; may mimic
acute abdomen
Abdominal tenderness/guarding Hypoglycemia
Back pain Reduced consciousness, delirium Hypercalcemia
Confusion
Most symptoms are nonspecific and present chronically, oen leading to delayed diagnosis.
Hyponatremia and, later, hyperkalemia are oen triggers to diagnosis, requiring biochemical
confirmation of adrenal insufficiency. Hyperpigmentation is a specific sign, but it is variably present
in individuals and must be compared with the patient's background pigmentation, such as that in
siblings. Adrenal crisis is a medical emergency with hypotension, marked acute abdominal symptoms,
and marked laboratory abnormalities, requiring immediate treatment. Continuing effort to prevent
adrenal crisis is integral to patient management. Additional symptoms and signs may arise from the
underlying cause of adrenal insufficiency—eg, associated autoimmune disorders, neurological features
of adrenoleukodystrophy, or disorders that may lead to adrenal infiltration.
Î ES recommends measurement of plasma ACTH to establish PAI. The
sample can be obtained at the same time as the baseline sample in
the corticotropin test or paired with the morning cortisol sample. In
patients with confirmed cortisol deficiency, a plasma ACTH >2x the
upper limit of the reference range is consistent with PAI. (1|⊕⊕⊕
)
Î ES recommends the simultaneous measurement of plasma renin and
aldosterone in PAI to determine the presence of mineralocorticoid
deficiency. (1|⊕⊕⊕
)
Î ES suggests that the etiology of PAI should be determined in all
patients with confirmed disease. (For diagnostic workup, see Table 2
and Figure 1.) (U)
