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Gadolinium-Associated Nephrogenic Systemic Fibrosis
Aka: Gadolinium-Associated Nephrogenic Systemic Fibrosis, Nephrogenic Fibrosing Dermopathy, Scleromyxedema-like Cutaneous Disease
- See Also
- Intravenous Contrast Related Acute Renal Failure
- Prevention of Kidney Disease Progression
- Chronic Kidney Disease
- Acute Kidney Injury
- Epidemiology
- Incidence:
- Rare, overall Incidence
- Severe Renal Failure and exposure to gadalinium: 4%
- Pathophysiology
- Triad of Gadolinium exposure, Renal disease, and Proinflammatory state
- Risk Factors
- Gadolinium Exposure
- Gadodiamide (Omniscan)
- Gadopentetate dimeglumine (Magnevist)
- Gadoversetamide (Optimark)
- Renal dysfunction (Stage 4-5)
- Severe renal dysfuntion (GFR<30 ml/min)
- Hemodialysis or Peritoneal Dialysis
- Acute Renal Failure
- Proinflammatory state
- Recent major surgery
- Thrombosis history
- Malignancy
- High dose Erythropoietin
- Prevention
- Avoid gadolinium-based Contrast Material when GFR <30 ml/min or Hepatorenal-mediated Acute Renal Failure
- Course
- Onset within 2-3 months of gadolinium exposure (median 11 days)
- Symptoms
- Pruritic rash involving symmetric extremities and trunk
- Signs
- Erythematous Plaques with induration and swelling symmetrically distributed on extremities and trunk
- Peau d'orange appearance
- Diagnosis
- Skin biopsy of lesion (include depth to subcutaneous fat or fascia)
- CD34 spindle-shaped fibrocytes with thickened collagen bundles
- Complications
- Debilitating joint contractures
- Multi-system fibrosis and resulting multi-system organ failure
- Respiratory Failure (due to diaphragmatic involvement)
- Prognosis
- Mortality: 31%
- References
- Cowper (2003) Curr Opin Rheumatol 15(6): 785-90 [PubMed]
- Sadowski (2007) Radiology 243(1): 148-57 [PubMed]
- Schlaudecker (2009) Am Fam Physician 80(7): 711-4 [PubMed]