Adrenal Insufficiency

Pathophysiology

• Classification: primary vs. secondary
• Primary
  • Primary adrenal insufficiency is also known as Addison's disease, named after a British physician who described the condition in 1856[1]
  • In the Western world, the most frequent cause of primary adrenal insufficiency is autoimmune adrenalitis
• Secondary
  • Pituitary/Hypothalamus failure
    • Given that aldosterone secretion by the adrenals does not depend primarily on ACTH, the zona glomerulosa continues to function appropriately in patients with secondary adrenal insufficiency. Mineralocorticoid deficiency is therefore present only in patients with primary adrenal insufficiency

Diagnosis and Evaluation

• Diagnosis of primary adrenal insufficiency is primarily made on clinical grounds, with a high index of suspicion given a patient’s history, physical and labs.

History and Physical

• Clinical manifestations of adrenal insufficiency[2]
  • Anorexia
  • Abdominal pain
  • Weakness
  • Weight loss
  • Fatigue
  • Hypotension
  • Salt craving
  • Hyperpigmentation of the skin in case of primary adrenal insufficiency

Labs

• Diagnosis is confirmed by measurements of morning serum cortisol and ACTH. Patients with primary adrenal insufficiency also exhibit abnormal aldosterone and renin levels.
• Confirmatory testing involves assessing the adrenal response to ACTH stimulation in the form of the corticotropin test.

Management

• Adrenal hormonal repletion
• Post-operative adrenal crisis
  • Any patient who has undergone ipsilateral partial or radical nephrectomy and is undergoing contralateral renal or adrenal surgery is at risk for a post-operative adrenal crisis. Obtaining old operative or pathology reports and examining cross-sectional imaging for the presence or absence of adrenal tissue are essential in this setting
    • In a patient with suspected post-operative adrenal crisis, consider 2mg dexamethasone or 100mg hydrocortisone

Questions

1. Which layer of the adrenal cortex continues to function in patients with secondary adrenal insufficiency?
2. List clinical manifestations of adrenal insufficiency

Answers

1. Which layer of the adrenal cortex continues to function in patients with secondary adrenal insufficiency?
2. List clinical manifestations of adrenal insufficiency

References

• Wein AJ, Kavoussi LR, Partin AW, Peters CA (eds): CAMPBELL-WALSH UROLOGY, ed 11. Philadelphia, Elsevier, 2015, chap 65