Ver el código de «Nephrogenic systemic fibrosis»

==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===
*[[SLE]]
*[[Hepatitis]]
*[[Eosinophilia]]

==Clinical Features==
[[File:NephrogenicSystemicFibrosis.png|thumb]]
*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==
*[[Scleroderma]], morphea
*[[Calciphylaxis]], which may coexist
*[[Cellulitis]]
*[[Necrotizing fasciitis]]
*[[Porphyria]]
*Eosinophilic fasciitis

{{Contrast complications DDX}}

==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==
*[[MRI contraindications]]

==External Links==

==References==
*Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.
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[[Category:Radiology]] 
[[Category:Renal]]