Updated: 8/25/2017

Gadolinium-Associated Nephrogenic Systemic Fibrosis

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Snapshot
  • A 56-year-old man presents to his primary care physician with muscle weakness and joint contractures in his hands. He reports to having developed a painful rash that feels hard and states he has a general “tightness” in his body.  He has a past medical history of type II diabetes mellitus and end-stage renal disease treated with hemodialysis. About 1 month prior to presentation, the patient had a transient ischemic attack and underwent an MRI with contrast of the brain. On physical exam, he has bilateral symmetric indurated papules and plaques on his forearms and shins, his hands have contractures, and his range of motion is limited in the involved joints. Laboratory evaluation reveals an elevated erythrocyte sedimentation rate, C-reactive protein, and ferritin.
Introduction
  • Clinical definition
    • rare systemic fibrosing disorder in patients with renal failure that is associated with gadolinium-containing contrast agent
      • primarily characterized by
        • thickening of skin
        • fibrosis of dermis, but can also affect muscle, fascia, lungs, and heart
  • Epidemiology
    • incidence
      • rare
    • demographics
      • occurs only in patients with renal failure
    • risk factors
      • renal failure
      • renal transplant
      • chronic kidney disease
      • exposure to gadolinium-containing agents
  • Etiology
    • gadolinium
      • an active component in contrast agents used in MRI studies
  • Pathogenesis
    • mechanism is unclear
      • gadolinium may dissociate from its chelating molecule and increase the exposure in patients with renal failure
        • gadolinium deposition in tissue may cause a tissue injury reaction
          • this may activate transforming growth factor TGF-β1 pathway
        • gadolinium may also directly increase the number of circulating fibrocytes by stimulating the bone marrow
          • increased number of fibrocytes can produce collagen and cause tissue fibrosis
  • Prognosis
    • 30% mortality
    • positive prognostic factors
      • recovery of renal function
Presentation
  • Symptoms
    • onset occurs 2-4 weeks after exposure to gadolinium but can occur up to years after exposure
    • skin lesions
      • start at extremities and move proximally to thighs and forearms
      • common distribution
        • ankles to below the knees
        • skin between wrists and upper arms
      • head is spared          
      • pruritic or painful
    • lung involvement
      • shortness of breath
  • Physical exam
    • skin lesions
      • symmetric and bilateral indurated papules and plaques
      • subcutaneous nodules
      • edematous
      • cobblestone or peau d’orange appearance
    • limited range of motion
    • joint contractures
    • sclerodactyly
Imaging
  • CT scan with contrast
    • indications
      • evaluation of systemic organ involvement
    • findings
      • fibrosis of fascia and muscles
Studies
  • Labs
    • ↑ erythrocyte sedimentation rate
    • ↑ C-reactive protein
    • ↑ ferritin
  • Biopsy reaching subcutaneous tissue
    • proliferation of CD34+ dermal fibrocytes
Differential
  • Scleroderma
    • presence of Raynaud phenomenon
Treatment
  • No medical intervention proven to be effective
  • The following may have some benefit
    • conservative
      • physical therapy
        • indications
          • for all patients to prevent or reverse immobility from disease
    • non-operative
      • extracorporeal photopheresis
        • indications
          • if disease progresses
      • ultraviolet A phototherapy
        • indications
          • if disease progresses
        • often used in combination with other therapies
Complications
  • Respiratory failure from lung involvement
  • Wheelchair dependence due to immobility
 

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