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Snapshot
  • A 25-year-old man presents to the clinic for an annual check-up. He is otherwise healthy and has no significant concerns. Basic laboratory studies demonstrate a sodium level of 129 mg/dL and potassium level of 5.9 mg/dL. A physical examination demonstrates hyperpigmentation of the oral mucosa. Review of systems were positive for generalized fatigue, 5-lbs unintentional weight loss, and some headaches.
Introduction
  • Clinical definition
    • disorder characterized by the loss of adrenal gland function leading to the deficiency of glucocorticoids, mineralocorticoids, and adrenal androgens
    • can be either acute or chronic depending of the etiology
  • Epidemiology
    • demographics
      • autoimmune causes (which accounts for 70-90% of cases in the U.S.) occur predominantly within the female population
    • risk factors
      • other autoimmune endocrinopathies  
  • Pathogenesis
    • any process that damages the adrenal cortices and leads to a deficiency of aldosterone, catecholamines, and cortisol
    • autoimmune adrenalitis (Addison disease)
      • most common cause in the U.S.
      • both humoral and cell-mediated immune mechanisms against the adrenal cortex
      • a small percentage of patients may have polyglandular autoimmune syndrome
    • infectious adrenalitis
      • tuberculosis (most common cause in the developing world)
      • disseminated fungal infections (e.g., histoplasmosis)
      • HIV infection
    • hemorrhagic infarction
      • Waterhouse-Friderichsen syndrome associated with meningococcemia (Neisseria meningitidis)
      • anticoagulant drug or heparin therapy (e.g., heparin-induced thrombocytopenia)
    • metastatic disease
      • commonly associated with lung, breast, and melanoma cancers
  • Associated conditions
    • approximately 1/2 of patients with autoimmune adrenal insufficiency have other autoimmune endocrine disorders (e.g., hypothyroidism)
Presentation
  • Symptoms 
    • fatigue  
    • weight loss  
    • nausea/vomiting
    • abdominal pain
    • muscle/joint pain
    • salt craving
    • adrenal crisis
  • Physical exam
    • skin and mucosal hyperpigmentation (if longstanding) 
      • due to increased production of proopiomelanocortin (POMC), a prohormone that is cleaved into ACTH and melanocyte-stimulating hormone (MSH)
    • hypotension
    • auricular-cartilage calcification
    • vitiligo
Imaging 
  • Computed tomography (CT)
    • may demonstrate bilateral adrenal injury, hemorrhage, or infarction 
Studies 
  • Serum cortisol concentration (e.g., morning serum cortisol)
    • best initial test
    • low serum cortisol (e.g., < 5 ug/dL) strongly suggests adrenal insufficiency 
  • Plasma ACTH concentration
    • best initial test but often not quickly available
    • high plasma ACTH concentration with low serum cortisol suggests primary adrenal insufficiency
  • ACTH stimulation test
    • will have low response to ACTH stimulation
  • Plasma aldosterone and renin levels
    • will have low aldosterone and high renin levels
  • Chemistry panel 
    • hyperkalemia
    • hyponatremia
Differential 
  • Secondary adrenal insufficiency
    • distinguishing factors
      • will not have hyperpigmentation on physical examination
      • will have low levels of ACTH
Treatment
  • Glucocorticoid replacement therapy
    • e.g., hydrocortisone or dexamethasone
    • stress doses indicated at times of stress (e.g., surgery)
    • significant adverse effects with chronic use (e.g., osteoporosis)
  • Mineralcorticoid replacement therapy
    • e.g., fludrocortisone
    • prevents sodium loss, intravascular volume depletion, and hyperkalemia
  • Androgen replacement therapy
    • e.g., dehydroepiandrosterone (DHEA)
    • appears to improve mood and psychological well-being
    • adverse effects include hirsutism, acne, and increased sweating/odor
Complications
  • Adrenal crisis
    • medical emergency managed with schedule IV glucocorticoid
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