Adrenal Insufficiency and Crisis
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Introduction
Worst is adrenal crisis, which can occur in people with adrenal insufficiency exposed to significant stress, including infection, trauma, surgery, and even pregnancy.
The Case of...
a simple case introducing clincial presentation and calling for a differential diagnosis to get students thinking.
Causes and Risk Factors
Primary adrenal insufficiency:
- autoimmune activity (most common; can be part of a polyglandular autoimmune syndrome)
- Addison's disease
- metastatic malignancy or lymphoma
- infarction or hemorrhage (can see bilaterally in pregnancy)
- infections: TB ,CMV, fungi
- infiltrative disorders: sarcoidosis, amyloidosis, hemochromatosis
- trauma, surgery
- drugs: ketoconazole, mitotane, aminoglutethimide, etomidate, cyproterone, rifampin, dilantin
- congenital adrenal hyperplasia, adrenoleukodystrophy, familiy glucocorticoid deficiency
Secondary, ACTH deficiency, can be caused by:
- chronic glucocorticoid therapy
- hypopituitarism (ie pituitary tumour)
Pathophysiology
Adrenal insufficiency becomes clinically manifest when more than 90% of the adrenal cortex is lost.
- Inadequate cortisol despite high ACTH levels
Congenital adrenal hyperplasia
- inadequate cortisol desipte high ACTH
- give synthetic GC to inhibit ACTH
Signs and Symptoms
Once treatment begins, signs and symptoms of excess replacement should also be monitored for.
- history
- physical exam
History
- weight loss
- fatigue (G)
- anorexia (G)
- nausea, vomiting, abdominal pain (G,M)
- salt craving (M)
- arthralgias, myalgias
- mental health
Crisis:
- GI
- weakness, apathy, confusion, decreased LOC, coma
- lightheadedness (result from decreased salt)
Excess:
Physical Exam
- hypotension
- auricular calcification
- increased skin pigmentation, especially of pressure areas, freckles, oral mucosa (ACTH breakdown pigments)
- pubic hair loss in women due to loss of androgens
Crisis:
- hypotension, shock
- dehydration
- fever
Excess:
- edema (excess G)
Investigations
- lab investigations
- diagnostic imaging
Lab Investigations
- hyponatremia and salt craving (M)
- hyperkalemia (mild)
- hypercalcemia
- mild acidosis
- hypoglycemia
AM cortisol (8-9 AM): cortisol levels are highest in morning, and the test is standardized as such. Greater than 270 is strong evidence that adrenal insufficiency is not present; below 100 is strong evidence that insuffiency is present.
ACTH stimulation tests can be done with serum cortisol measured 30 minutes after injection, but can be false negative in 10-15% of patients. 250 mcg is of ACTH is administered.
An insulin stress test should cause hypoglycemia and a spike in both plasma cortisol and serum growth hormone within 1-2 hours.
- hypoglycemia
- hyponatremia, hyperkalemia
Diagnostic Imaging
Differential Diagnosis
Treatments
Hydrocortisone is the active hormone and the preferred treatment, as it has a shorter half-life. It is given BID, with a greater dose in the AM. Correct dosing can be assessed by measuring urinary free cortisol and the day curve.
Other options include prednisone, cortisone acetate, or dexamathasone.
Fludrocortisone is a synthetic mineralocorticoid given as well to people with primary insufficiency.
Stress dosing is needed for infections, illnesses, etc. Double the dose for three days and then reevaluate.
Patients should also have medic alert bracelets letting them know
Emergency kits are also necessary for if patients can't keep their PO cortisol down - an injectable amount...
Adrenal crisis is an emergency - treat as soon as it is suspected!
5 S's
- salt, sugar, steroids, support, and search for precipitating illness
volume replacement with D5W
give 100 mg hydrocortisone IV Q6H
Consequences and Course
Resources and References
Topic Development
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