Addison Disease

Question 1

What is Addison’s disease?

Answer 1

• Characterized by a deficiency of cortisol, androgens and aldosterone as a result of adrenal cortices destruction
• Clinical presentation of adrenal insufficiency is variable, dependent upon whether onset is acute (leading to adrenal crisis) or chronic, with symptoms more insidious and vague.

Question 2

What causes Addison’s disease?

Answer 2

• Rare, occurring in an average 50:1 million patients
• Female to male ratio 2:1
• Idiopathic autoimmune- MOST COMMON cause for Addison Disease; some patients may also have other related autoimmune disorders such as diabetes Type 1, and hypothyroidism
• Sudden withdrawal of long term exogenous glucocorticoids (asthma, transplantation, inflammatory disease) is MOST common cause of Adrenal crisis
• Exogenous glucocorticoids - prolonged administration of pharmacologic doses of synthetic glucocorticoids cause corticotropic (ACTH) deficiency and consequently adrenal insufficiency
• Extreme stress
• Trauma
• Adrenal hemorrhage, post adrenalectomy
• Sepsis
• Tuberculosis – world-wise cause

Question 3

What are the signs of adrenal crisis?

Answer 3

• Marked and rapid worsening of chronic findings
• Dehydration, hypotension, or shock out of proportion to severity of current illness and unresponsive to IV fluids (fluid resuscitation)
• N/V
• anorexia, lethargy
• abdominal pain- acute abdomen
• unexplained hypoglycemia
• unexplained fever
• hyponatremia, hyperkalemia, azotemia, hypercalcemia, eosinophilia
• other autoimmune endocrine deficiencies, such as hypothyroidisms or gonadal failure
• confusion, mental status changes, stupor, convulsions

Question 4

What are signs of chronic adrenal insufficiency?

Answer 4

• Anorexia
• GI: Nausea/vomiting, abdominal pain
• Postural dizziness
• Salt craving
• Weakness/fatigue/progressive weakness
• Muscle or joint pains
• Sparse axillary hair
• Weight loss
• Hypotension
• hyperpigmentation in the buccal mucosa and skin creases r/t excess ACTH
  
  • apparent in knuckles, knees, posterior neck, elbows and palmar creases
  • signifies a deficiency in cortisol, not in ACTH

Question 5

What lab/diagnostic tests do you order for a patient with Addison’s disease?

Answer 5

• Hyponatremia – MOST COMMON INITIAL laboratory finding
• Hyperkalemia
• Hypoglycemia
• Elevated erythrocyte sedimentation rate
• Neutropenia < 5000 /microL
• Eosinophil count > 300/microL
• Lymphocytosis – in approx. 50% of patients
• Plasma cortisol < 5 mg per deciliter at 8am
• Hypercalcemia – may be present
• Elevated BUN – secondary to decreased extracellular fluid volume caused by aldosterone deficiency
• Metabolic acidosis – secondary to hypotension, decreased renal function, and decreased hydrogen ion excretion due to aldosterone deficiency
• Synthetic ACTH test (simplified cosyntropin test)
  
  • Cosyntropin 0.25mg IV is administered. **Discontinue hydrocortisone at least 8 hours prior to test.
  • Plasma cortisol levels are obtained 30-60 minutes post administration
  • Normally cortisol levels should rise 20 micrograms/dl or higher
  • If no rise in ACTH levels, test is diagnostic for primary adrenal disease.
• Chest x-ray/ Abdominal x-ray
• CT scan as warranted

Question 6

How do you manage a patient with Addison’s disease as an outpatient?

Answer 6

• Endocrinology consult/referral
• Glucocorticoid and Mineral corticoid replacement therapy as indicated prn
  
  • Hydrocortisone 10-15 mg po am and 5-10 mg po in afternoon q day
  • If additional therapy is needed, fludrocortisone acetate may be initiated.
  • Educate patient on signs and symptoms of adrenal crisis.
  • Patients should wear medic alert bracelets
• Dosage may need to be adjusted (increased) during times of stress- including infections, surgery, major illnesses, etc.

Question 7

Describe the acute management of a patient with Addison’s disease

Answer 7

• Endocrinology consult
• Once diagnosis made, give:
  
  • Hydrocortisone (Solu-cortef) 100-300mg IV with 0.9NS for fluid replacement (resuscitation)
• After initial administration, give:
  
  • Hydrocortisone phosphate or hydrocortisone sodium succinate 100mg IV q 6 hours until patient is stable, then decrease to hydrocortisone 50mg q 6 hours IV
  • By day 4 or 5, taper to maintenance therapy and add mineral corticoid therapy prn
  • Initiate potassium replacement therapy.
  • Volume may also be replaced with D5 45NS after initially replaced with 0.9NS
  • Change patient to po therapy
  • Treat underlying cause.