Nephrogenic systemic fibrosis: Difference between revisions
No edit summary |
No edit summary |
||
| Line 31: | Line 31: | ||
==Treatment== | ==Treatment== | ||
*Treatments | *Treatments anecdotal and of minimal benefit | ||
**Extracorporeal photopheresis (ECP) | **Extracorporeal photopheresis (ECP) | ||
**UV phototherapy | **UV phototherapy | ||
| Line 42: | Line 42: | ||
*Does not inherently require inpatient care | *Does not inherently require inpatient care | ||
== | ==See Also== | ||
*[[MRI contraindications]] | |||
==References== | |||
*Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview. | *Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview. | ||
<references/> | <references/> | ||
[[Category: | [[Category:Rads]] | ||
Revision as of 00:18, 3 May 2016
Background
- Gadolinium exposure (MRI with contrast) to pts 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
- Hx 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
- 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
Treatment
- 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.