Nephrogenic systemic fibrosis: Difference between revisions

(2 intermediate revisions by the same user not shown)
Line 1: Line 1:
==Background==
==Background==
*Gadolinium exposure (MRI with contrast) to patients with renal insufficiency (HD, PD, transplant)
*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===
Line 15: Line 14:
*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
**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)
Line 54: Line 48:


==Disposition==
==Disposition==
*Discharge (Does not inherently require inpatient care)
*Discharge (does not inherently require inpatient care)
*Follow up with dermatologist for deep skin bx
*Follow up with dermatologist for deep skin biopsy
*Follow up with nephrologist for renal disease management
*Follow up with nephrologist for renal disease management



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