Nephrogenic systemic fibrosis

From WikEM
Jump to: navigation, search

Background

  • Rare but serious scleroderma-like condition associated with gadolinium (MRI contrast) exposure in patients with renal impairment (e.g. hemodialysis, peritoneal dialysis, transplant)
    • Risk with any renal impairment, acute or chronic, does not have to have ESRD
  • Poorly understood pathogenesis
  • Chronic, progressive condition with rare cases of resolution in cases of return of renal function

Associated Illnesses

Clinical Features

Error creating thumbnail: File missing
  • History of HD, PD, renal transplant
  • Gadolinium exposure, with timeline of symptom onset variable
    • 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
    • Peau d'orange appearance
    • Extremities most commonly affected, followed by trunk. Face rarely involved
  • Flexion contractures when lesions overlie joints, limited range of motion
    • Hand stiffening
    • Extremities more commonly affected than trunk
  • Neuromuscular involvement can occur
  • In most severe cases, fibrosis involves internal organs (heart, lungs, liver)

Differential Diagnosis

Evaluation

  • No particular lab or imaging diagnostics
  • Deep skin biopsy, including dermis, subQ fat, fascia

Management

  • No specific management, supportive care
  • Treatments anecdotal and of minimal benefit
    • Extracorporeal photopheresis (ECP)
    • UV phototherapy
    • Immunotherapy
  • No surgical role except renal transplant

Disposition

  • Discharge (does not inherently require inpatient care)
  • Follow up with dermatologist for deep skin biopsy
  • Follow up with nephrologist for renal disease management

See Also

External Links

References