Nephrogenic systemic fibrosis: Difference between revisions
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[[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== | |||
==References== | |||
<references/> | |||
[[Category:Renal]] [[Category:Radiology]] | |||
Revision as of 06:19, 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
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
- Scleroderma, morphea
- Calciphylaxis, which may coexist
- Cellulitis
- Necrotizing fasciitis
- Porphyria
- Eosinophilic fasciitis
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
- Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.
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