Nephrogenic Systemic Fibrosis

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Background

  • Rare but serious scleroderma-like condition associated with gadolinium (MRI contrast) exposure in patients with renal impairment
  • Risk of nephrogenic systemic fibrosis (NSF) with any renal impairment, acute or chronic, does not have to have ESRD

Clinical Features

  • Develops days to years after gadolinium exposure
  • Chronic, progressive
  • Skin becomes tight, thick, shiny, and indurated skin, with fibrotic nodules and plaques and brawny hyperpigmentation
    • Painful, pruritic
    • Extremities most commonly affected, followed by trunk. Face rarely involved
  • Flexion contractures when lesions overlie joints, limited range of motion
  • Neuromuscular involvement can occur
  • In most severe cases, fibrosis involves internal organs (heart, lungs, liver)

Differential Diagnosis

Evaluation

Management

  • No specific management, supportive care

Disposition

  • Discharge

See Also

External Links

References