Gadolinium-Associated Nephrogenic Systemic Fibrosis

1. Epidemiology
   1. Incidence:
      1. Rare, overall Incidence
      2. Severe Renal Failure and exposure to gadalinium: 4%
2. Pathophysiology
   1. Triad of Gadolinium exposure, Renal disease, and Proinflammatory state
3. 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
4. Prevention
   1. Avoid gadolinium-based Contrast Material when GFR <30 ml/min or Hepatorenal-mediated Acute Renal Failure
5. Course
   1. Onset within 2-3 months of gadolinium exposure (median 11 days)
6. Symptoms
   1. Pruritic rash involving symmetric extremities and trunk
7. Signs
   1. Erythematous Plaques with induration and swelling symmetrically distributed on extremities and trunk
   2. Peau d'orange appearance
8. Diagnosis
   1. Skin biopsy of lesion (include depth to subcutaneous fat or fascia)
      1. CD34 spindle-shaped fibrocytes with thickened collagen bundles
9. Complications
   1. Debilitating joint contractures
   2. Multi-system fibrosis and resulting multi-system organ failure
   3. Respiratory failure (due to diaphragmatic involvement)
10. Prognosis
    1. Mortality: 31%
11. References
    1. Cowper (2003) Curr Opin Rheumatol 15(6): 785-90
    2. Sadowski (2007) Radiology 243(1): 148-57
    3. Schlaudecker (2009) Am Fam Physician 80(7): 711-4