Difference between revisions of "Nephrogenic systemic fibrosis"

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==Background==
 
==Background==
*Gadolinium exposure (MRI with contrast) to patients with renal insufficiency (HD, PD, transplant)
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*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
 
*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
 
  
 
===Associated Illnesses===
 
===Associated Illnesses===
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*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
*Tightened, thickened, shiny skin
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**Develops days to years after gadolinium exposure
*Peau d'orange appearance
 
*Hand stiffening
 
*Flexion contractures, severe mobility issues
 
*Extremities more commonly affected than trunk
 
*Face is almost never involved
 
 
 
 
 
*Develops days to years after gadolinium exposure
 
 
*Chronic, progressive
 
*Chronic, progressive
 
*Skin becomes tight, thick, shiny, and indurated skin, with fibrotic nodules and plaques and brawny hyperpigmentation
 
*Skin becomes tight, thick, shiny, and indurated skin, with fibrotic nodules and plaques and brawny hyperpigmentation
 
**Painful, pruritic
 
**Painful, pruritic
 +
**Peau d'orange appearance
 
**Extremities most commonly affected, followed by trunk. Face rarely involved
 
**Extremities most commonly affected, followed by trunk. Face rarely involved
 
*Flexion contractures when lesions overlie joints, limited range of motion
 
*Flexion contractures when lesions overlie joints, limited range of motion
 +
**Hand stiffening
 +
**Extremities more commonly affected than trunk
 
*Neuromuscular involvement can occur
 
*Neuromuscular involvement can occur
 
*In most severe cases, fibrosis involves internal organs (heart, lungs, liver)
 
*In most severe cases, fibrosis involves internal organs (heart, lungs, liver)
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==Disposition==
 
==Disposition==
*Discharge (Does not inherently require inpatient care)
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*Discharge (does not inherently require inpatient care)
*Follow up with dermatologist for deep skin bx
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*Follow up with dermatologist for deep skin biopsy
 
*Follow up with nephrologist for renal disease management
 
*Follow up with nephrologist for renal disease management
  

Latest revision as of 06:28, 24 September 2016

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

NephrogenicSystemicFibrosis.png
  • 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