Nephrogenic systemic fibrosis

Revision as of 04:06, 27 June 2016 by Neil.m.young (talk | contribs) (Text replacement - "==Differential==" to "==Differential Diagnosis==")

Background

  • Gadolinium exposure (MRI with contrast) to patients with renal insufficiency (HD, PD, transplant)
  • Poorly understood pathogenesis
  • Chronic, progressive condition with rare cases of resolution in cases of return of renal function

Clinical Features

  • Hx of HD, PD, renal transplant
  • Gadolinium exposure, with timeline of symptom onset variable
  • Tightened, thickened, shiny skin
  • Peau d'orange appearance
  • Hand stiffening
  • Flexion contractures, severe mobility issues
  • Extremities more commonly affected than trunk
  • Face is almost never involved
  • Associations:

Differential Diagnosis

Workup

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

Treatment

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

Disposition

  • Dermatologist for deep skin bx
  • Nephrologist for renal disease management
  • Does not inherently require inpatient care

See Also

References