Nephrogenic systemic fibrosis: Difference between revisions

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*Poorly understood pathogenesis
*Poorly understood pathogenesis
*Chronic, progressive condition with rare cases of resolution in cases of return of renal function
*Chronic, progressive condition with rare cases of resolution in cases of return of renal function
*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==
==Clinical Features==
[[File:NephrogenicSystemicFibrosis.png|thumb]]
*History 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
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**[[Hepatitis]]
**[[Hepatitis]]
**Eosinophilia
**Eosinophilia
*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==
==Differential Diagnosis==
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==Management==
==Management==
*No specific management, supportive care
*Treatments anecdotal and of minimal benefit
*Treatments anecdotal and of minimal benefit
**Extracorporeal photopheresis (ECP)
**Extracorporeal photopheresis (ECP)
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==Disposition==
==Disposition==
*Dermatologist for deep skin bx
*Discharge (Does not inherently require inpatient care)
*Nephrologist for renal disease management
*Follow up with dermatologist for deep skin bx
*Does not inherently require inpatient care
*Follow up with nephrologist for renal disease management


==See Also==
==See Also==
*[[MRI contraindications]]
*[[MRI contraindications]]


==References==
*Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.
<references/>
[[Category:Radiology]]
[[File:NephrogenicSystemicFibrosis.png|thumb]]
==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==
*[[MRI contraindications]]
==External Links==
==External Links==


==References==
==References==
*Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.
<references/>
<references/>


[[Category:Renal]] [[Category:Radiology]]
[[Category:Radiology]]  
[[Category:Renal]]

Revision as of 06:21, 24 September 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
  • 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

NephrogenicSystemicFibrosis.png
  • 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:


  • 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

Workup

  • 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 bx
  • Follow up with nephrologist for renal disease management

See Also

External Links

References