Nephrogenic systemic fibrosis
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Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy) is a scleroderma-like fibrosing condition believed to be caused by gadolinium toxicity as it is associated with the stability of gadolinium contrast agents.
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Histiopathology
Diagnosed by skin biopsy[1].
- Dermal and subdermal thickening
- Abundant collagen fibers and mucin deposits
- Marked increase in spindle-shaped fibroblastic cells
Risk factors
Risk factors appear to include:
- Exposure to gadolinium contrast agents17547734[2]
- Renal insufficiency (estimated glomerular filtration rate [eGFR] < 60 mL/min/1.73 m2[3]
- Proinflammatory events[4] such as:
- Major surgery
- Infection
- Vascular events
Clinical presentation
It is associated clinically with:
- Erythematous to browny skin induration with a peau d'orange appearance, plaques, nodules, and papules[5]
- Yellow scleral plaques
- Antiphospholipid antibodies[6]
- Raised erythrocyte sedimentation rate
- Raised C-reactive protein
It can cause:
External Links
- Case 35-2004 — A 68-Year-Old Man with End-Stage Renal Disease and Thickening of the Skin N Engl J Med. 2004;351:2219-2227 - Registration required
References
- ↑ Piera-Velázquez S, Sandorfi N, Jiménez SA. Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy: clinical aspects.Skinmed. 2007;6(1):24-7
- ↑ Sadowski EA, Bennett LK, Chan MR, Wentland AL, Garrett AL, Garrett RW, et al. Nephrogenic systemic fibrosis: risk factors and incidence estimation. Radiology 2007;243(1):148-57. (Direct link – subscription may be required.)
- ↑ Sadowski EA, Bennett LK, Chan MR, Wentland AL, Garrett AL, Garrett RW, et al. Nephrogenic systemic fibrosis: risk factors and incidence estimation. Radiology 2007;243(1):148-57. (Direct link – subscription may be required.)
- ↑ Sadowski EA, Bennett LK, Chan MR, Wentland AL, Garrett AL, Garrett RW, et al. Nephrogenic systemic fibrosis: risk factors and incidence estimation. Radiology 2007;243(1):148-57. (Direct link – subscription may be required.)
- ↑ Piera-Velázquez S, Sandorfi N, Jiménez SA. Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy: clinical aspects.Skinmed. 2007;6(1):24-7
- ↑ Cowper SE. Nephrogenic fibrosing dermopathy: the first 6 years. Current opinion in rheumatology 2003;15(6):785-90.
- ↑ Cowper SE. Nephrogenic fibrosing dermopathy: the first 6 years. Current opinion in rheumatology 2003;15(6):785-90.
- ↑ Gibson SE, Farver CF, Prayson RA. Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature. Archives of pathology & laboratory medicine 2006;130(2):209-12.
