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Addison Disease

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Topic updated on 04/12/17 2:33pm

Introduction
  • A disorder caused by the destruction of the adrenal cortices
    • involves all three layers of the cortex
    • results in ↓ production of glucocorticoid (cortisol), mineralcorticoids (aldosterone), and steroids
  • Autoimmune destruction accounts for 80% of the spontaneous cases
    • other causes include:
      • congenital enzyme deficiencies
      • TB
      • AIDS
      • metastasis
  • Associated with other autoimmune conditions
Presentation
  • Symptoms  
    • fatigue worsened by stress
    • weakness
    • weight loss
    • nausea and vomitting
  • Physical exam
    • increased skin pigmentation
      • due to melanocyte stimulating hormone, a by-product of ↑ ACTH
        • ACTH/MSH both arise from pro-opiomelanocortin (POMC)
      • most commonly seen in mouth
    • hypotension
      • due to ↓ production of mineralcorticoids (aldosterone)
        • results in hyponatremic volume contraction
Evaluation
  • Labs
    • must determine if ↓ production of cortisol is primary (of adrenal orgin) or secondary (of pituitary origin)
      • ↑ plasma ACTH (primary)
      • plasma ACTH (secondary)
    • ACTH stimulation test
      • low cortisol and 17-OH levels in response
    • metyrapone test
      • ↑ ACTH without ↑ in 11-deoxycortisol
    • hyponatremia and hyperkalemia
      • due to ↓ aldosterone that results in decreased sodium reapsorption and decreased potassium secretion
    • hypoglycemia
      • due to ↓ cortisol-stimulated gluconeogenesis
    • metabolic acidosis
      • due to bicarbonate
    • eosinophilia, lymphocytosis, and neutropenia
      • due to ↓ cortisol
        • normally suppresses levels of eosinophils and lymphocytes
        • normally de-marginates a portion of the neutrophil population which results in ↑ measurable neutrophil count
Treatment
  • Replace
    • glucocorticoids
    • mineralocorticoids
  • Administer stress dose steroids at time of stress (surgery or significant illness)


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