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Updated: Jan 5 2021

Cushing Syndrome

  • Snapshot
    • A 42-year-old obese woman who does not smoke presents with diastolic hypertension and menstrual irregularities. Physical exam shows a full, plethoric-appearing face, increased facial hair, truncal obesity, and purple striae around the abdomen. Scattered echymoses are present over the entire body. Labs show a Hgb of 18 g/dL and a WBC of 18,000/mm^3. The leukocyte differential shows an absolute neutrophillic leukocytosis. CXR is normal.
  • Introduction
    • A condition that refers to the manifestations of hypercortisolism
      • results in hyperplasia of the adrenal cortex
        • specifically the fasiculata
    • There are several types
      • iatrogenic Cushing's
        • patients taking steroids is the most common cause of Cushing's syndrome
      • pituitary adenoma (Cushing's disease)
        • most common pathogenic cause (70%)
        • majority of adenomas are benign
        • cause bilateral hyperplasia of the adrenal glands
          • associated increase in conversion of norepinephrine to epinephrine in medulla
            • enzyme involved is phenylethanolamine n-methyltransferase
      • adrenal Cushing's
        • adenoma of the adrenals
      • ectopic Cushing's
        • ectopic ACTH secretion
        • extremely high ACTH
        • most commonly from small cell carcinoma of the lung
          • less commonly thymic cancer
  • Presentation
    • Symptoms
      • depression and psychological changes
      • oligomenorrhea
      • growth retardation
      • weakness
        • catabolism of muscle for gluconeogenesis
      • symptoms of diabetes (polydipsia, polyuria, and dysuria)
    • Physical exam
      • diastolic hypertension
      • central obesity
      • muscle wasting
      • thin skin that easily bruises/purple abdominal striae
        • due to weakening of collagen
      • hirsutism
      • moon facies
      • buffalo hump
  • Evaluation
    • Labs
      • hyperglycemia
        • cortisol is gluconeogenic
      • hypokalemia
        • at high concentrations cortisol can have partial activity at the aldosterone receptor
      • screen for 24-hour free urinary cortisol as well as a serum cortisol level
        • high positive and negative predictive value
      • serum ACTH to localize lesion
        • iatrogenic
          • ↓ ACTH
        • pituitary
          • ↑ ACTH
        • adrenal
          • ↓ ACTH
        • ectopic
          • ↑ ACTH
      • if ACTH is high, then use high dose dexamethasone suppression test
        • pituitary
          • ↓ cortisol production (i.e. production is suppressible)
        • ectopic
          • no change in cortisol (i.e. production is NOT suppressible)
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