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Gadolinium-Associated Nephrogenic Systemic Fibrosis

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Gadolinium-Associated Nephrogenic Systemic Fibrosis, Nephrogenic Fibrosing Dermopathy, Scleromyxedema-like Cutaneous Disease

  • Epidemiology
  1. Incidence:
    1. Rare, overall Incidence
    2. Severe Renal Failure and exposure to gadalinium: 4%
  • Pathophysiology
  1. Triad of Gadolinium exposure, Renal disease, and Proinflammatory state
  • Risk Factors
  1. Gadolinium Exposure
    1. Gadodiamide (Omniscan)
    2. Gadopentetate dimeglumine (Magnevist)
    3. Gadoversetamide (Optimark)
  2. Renal dysfunction (Stage 4-5)
    1. Severe renal dysfuntion (GFR<30 ml/min)
    2. Hemodialysis or Peritoneal Dialysis
    3. Acute Renal Failure
  3. Proinflammatory state
    1. Recent major surgery
    2. Thrombosis history
    3. Malignancy
    4. High dose Erythropoietin
  • Prevention
  1. Avoid gadolinium-based Contrast Material when GFR <30 ml/min or Hepatorenal-mediated Acute Renal Failure
  • Course
  1. Onset within 2-3 months of gadolinium exposure (median 11 days)
  • Symptoms
  1. Pruritic rash involving symmetric extremities and trunk
  • Signs
  1. Erythematous Plaques with induration and swelling symmetrically distributed on extremities and trunk
  2. Peau d'orange appearance
  • Diagnosis
  1. Skin biopsy of lesion (include depth to subcutaneous fat or fascia)
    1. CD34 spindle-shaped fibrocytes with thickened collagen bundles
  • Complications
  1. Debilitating joint contractures
  2. Multi-system fibrosis and resulting multi-system organ failure
  3. Respiratory Failure (due to diaphragmatic involvement)
  • Prognosis
  1. Mortality: 31%