Nephrogenic Systemic Fibrosis
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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
