Difference between revisions of "Nephrogenic systemic fibrosis"

Line 3: Line 3:
 
*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
Line 17: Line 20:
 
**[[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==
Line 31: Line 44:
  
 
==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)
Line 38: Line 53:
  
 
==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