Difference between revisions of "Nephrogenic systemic fibrosis"

(Text replacement - "==Treatment==" to "==Management==")
(Text replacement - "Hx " to "History ")
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==Clinical Features==
 
==Clinical Features==
*Hx of HD, PD, renal transplant
+
*History of HD, PD, renal transplant
 
*Gadolinium exposure, with timeline of symptom onset variable
 
*Gadolinium exposure, with timeline of symptom onset variable
 
*Tightened, thickened, shiny skin
 
*Tightened, thickened, shiny skin

Revision as of 05:35, 15 July 2016

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

  • History 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

Management

  • 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