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45 Cards in this Set

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What are the 3 forms of adrenal insufficiency?
1. primary
2. secondary
3. tertiary
What is another name for primary adrenal insufficiency?
Addison's disease; due to destruction of both adrenal glands
What percentage of the adrenal glands must be destroyed before clinical signs of insufficiency appear?
Greater than 90%
What is the most common cause of primary adrenal gland destruction worldwide?
TB
What is the most common cause of primary adrenal gland destruction in the US?
autoimmune (often seen as part of a polyglandular deficiency syndrome)
What is type 1 polyglandular deficiency syndrome?
hypoparathyroidism and mucocutaneous candidiasis
What is type 2 polyglandular deficiency syndrome?
autoimmune thyroiditis and type 1 DM
What is secondary adrenal insufficiency?
decreased ACTH production due to disruption of pituitary function
What is tertiary adrenal insufficency?
It is the most common cause of adrenal insufficiency in the US. It is due to exogenous steroid use, which suppresses but does not destroy the gland.
How can tertiary adrenal insufficiency occur?
1. Following steroid discontinuation if the steroid is not tapered appropriately.
2. It can also occur despite patient's usual steroid dose if the body is under great stress and the suppressed adrenals are unable to respond.
A 65 yo woman on chronic steroid for RA develops PNA. She presents with hypotension and tachycardia. What may be happening?
The patient could be septic. But this can also be a common presentation for adrenal insufficiency.
What types of adrenal excess are there?
1. adrenal hypertrophy
2. adrenal adenoma
3. adrenal carcinoma

These all can produce excess aldosterone, cortisol (Cushing's syndrome) or catecholamines (pheochromocytoma).
What is Cushing's syndrome?
A state of chronic glucocorticoid excess from any source.
What is the most common cause of Cushing's syndrome?
Long-term steroid use
What is Cushing's disease caused by?
A pituitary adenoma secreting ACTH. This is the second most common cause of Cushing's syndrome.
What are some of the less common causes of Cushing's syndrome?
1. adrenal hyperplasia
2. adrenal adenoma
4. ectopic ACTH production, usually from small cell lung cancer.
What are the 3 most common causes of adrenal insufficiency?
1. autoimmune disease
2. steroid use (tertiary)
3. TB
What are some uncommon causes of adrenal insufficiency?
1. bilateral adrenal hemorrhage
2. fungal infection
3. CMV adrenalitis (AIDS patients)
4. lymphoma
5. metastatic cancer
6. pituitary failture (secondary)
How does chronic adrenal insufficiency present in the ambulatory setting?
There are usually nonspecific complaints:
1. fatigue
2. arthralgias
3. myalgias
4. vague abdominal complaints
5. episodes of volume depletion or hypotension
How does acute adrenal insufficiency usually present clinically?
acute adrenal insufficiency (aka adrenal crisis), often occurs with cardiovascular collapse mimicking sepsis (hypotension, orthostasis)
Acute adrenal insufficiency is often preciptated by what types of conditions?
gastroenteritis, viral illness, bacterial infection or some other systemic stress.
Why does a patient get hyperpigmented from chronic primary adrenal gland failure?
The compensating pituitary overproduces ACTH and a byproduct, melanocyte stimulating hormone (MSH), which results in diffuse hyperpigmentation accentuated in the palmar creases and gums
Does secondary adrenal insufficiency present with hyperpigmentation?
No, because the pituitary is producing neither ACTH nor MSH.
In terms of signs and symptoms, how is primary adrenal insufficiency different from secondary insufficiency?
hypotension, orthostasis and hyperpigmentation occurs with primary adrenal insufficiency, not in secondary adrenal insufficiency where mineralcorticoid levels are preserved.
True or False: Secondary adrenal insufficiency due to pituitary failure usually presents with similarly fatigue, arthralgias/myalgias, but the abdominal pain and hypotension are less prominent because mineralocorticoid production is preserved.
True
What lab test do you obtain to diagnose adrenal insufficiency?
Get a random cortisol level. A level greater than 20 ug/dL rules out adrenal insufficiency.
Poorly controlled hypertension can arise from excess aldosterone, cortisol or catecholamine. In what types of patients should you suspect adrenal excess?
1. Very young hypertensive patients
2. Patients with difficult-to-control hypertension
What types of lab abnormalities would you see in adrenal excess?
1. hypokalemia (too much aldo)
2. hypernatremia (too much aldo)
3. metabolic alkalosis (shifting from potassium?)
*these can occur with elevated aldosterone or cortisol.
What are some physical clues to excess cortisol?
cushingoid appearance with facial plethora, central obesity, hirsutism and proximal muscle wasting.
What types of signs and symptoms does a pheochromocytoma present with?
1. episodic severe hypertension
2. sweating
3. flushing
4. headaches
T or F: Some pheochromocytomas are associated with neurofibromatosis or medullary thyroid cancer.
True
How do patients with Cushing's syndrome present?
Consider the diagnosis in patients who look "cushingoid" because patients rarely complain of symptoms.
How do I diagnose Cushing's syndrome?
1. Screen for cortisol excess by a 24 hour urine cortisol (normal less than 100 ug/24hours). Levels greater than 250 ug/day diagnose Cushing's syndrome, when levels less than 65 exclude the diagnosis.
When do false positives occur in the 24 hour urine cortisol test for Cushing's syndrome?
False-positive tests occur with depression, anorexia, stress, alcoholism and oral contraceptive use.
How do I work-up patients for hyperaldosteronism?
You have two options:
1. Do a serum aldosterone-to-renin ratio test. Aldosterone greater than 20 ng/dL and a ratio greater than 100 (with renin units in ng/ml/three hours) are highly specific for the diagnosis.
2. Do a 24 hour urine aldosterone collection. An aldosterone collection of greater than 20 ug in the setting of low plasma renin less than 5 ug/dl makes the diagnosis.
When should I suspect a pheochromocytoma?
Suspect pheos with presence of the 5 Hs (each usually episodic):
Hypertension
Headache
Hyperhidrosis
Hypotension (orthostatic)
Hyperglycemia
How do you screen for a pheo?
Get a 24 hour urine collection of catecholamines, metanephrines or vanillylmandelic acid. If screening is positive, obtain an abdominal MRI to look for the source.
How do I treat adrenal insufficiency?
In pat
How do you treat patients with suspected primary adrenal insufficiency and hemodynamic instability?
1. Give dexamethasone 4 mg IV immediately, which will not interfere with subsequent urine testing OR hypdrocortisone 100m mg IV q eight hours.
2. Search for precipitating infection or other physical source of stress
What do you use as maintenance therapy for chronic adrenal insufficiency?
Dexamethasone 0.5 mg or prednisone 5 mg qhs.

Alternatively, give hydrocortisone 15 mg qam and 5-10 mg in early afternoon.

Mineralcorticoid replacement for patients with primary adrenal insufficiency is with fludrocortisone 0.1 mg qd.
What do you do for patients with presumed tertiary insufficiency on long-term exogenous corticosteroids, when they are about to undergo a stressful event such as surgery or have an acute illness?
Current practice includes hydrocortisone 100 mg IV on call to the OR, or 50-1000 mg IV q8 h for 24 hours, then rapidly tapered when the patient stabilizes.
Treating adrenal excess syndromes: When aldosterone, cortisol or catecholamine excess is due to an adrenal tumor, what do you do?
surgical removal
How should you prepare a patient pre-operatively if he or she is getting a pheochromcytoma is being removed?
Give an alpha and beta blockade to avoid life-threatening catecholamine bolus.
How can you treat aldosterone excess due to adrenal hyperplasia?
1. Surgical removal
2. Lifelong spironolactone therapy
How do you treat Cushing's disease due to pituitary or ectopic ACTH production?
This requires surgical removal of the source of ACTH.