Nephrogenic systemic fibrosis: Difference between revisions
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==Background== | ==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 | *Poorly understood pathogenesis | ||
*Chronic, progressive condition with rare cases of resolution in cases of return of renal function | *Chronic, progressive condition with rare cases of resolution in cases of return of renal function | ||
===Associated Illnesses=== | |||
*[[SLE]] | |||
*[[Hepatitis]] | |||
*[[Eosinophilia]] | |||
==Clinical Features== | ==Clinical Features== | ||
* | [[File:NephrogenicSystemicFibrosis.png|thumb]] | ||
*History of HD, PD, renal transplant | |||
*Gadolinium exposure, with timeline of symptom onset variable | *Gadolinium exposure, with timeline of symptom onset variable | ||
* | **Develops days to years after gadolinium exposure | ||
*Peau d'orange appearance | *Chronic, progressive | ||
* | *Skin becomes tight, thick, shiny, and indurated skin, with fibrotic nodules and plaques and brawny hyperpigmentation | ||
**Painful, pruritic | |||
*Extremities | **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== | ==Differential Diagnosis== | ||
| Line 26: | Line 34: | ||
*Eosinophilic fasciitis | *Eosinophilic fasciitis | ||
== | {{Contrast complications DDX}} | ||
==Evaluation== | |||
*No particular lab or imaging diagnostics | *No particular lab or imaging diagnostics | ||
*Deep skin biopsy, including dermis, subQ fat, fascia | *Deep skin biopsy, including dermis, subQ fat, fascia | ||
== | ==Management== | ||
*No specific management, supportive care | |||
*Treatments anecdotal and of minimal benefit | *Treatments anecdotal and of minimal benefit | ||
**Extracorporeal photopheresis (ECP) | **Extracorporeal photopheresis (ECP) | ||
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==Disposition== | ==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== | ==See Also== | ||
*[[MRI contraindications]] | *[[MRI contraindications]] | ||
==External Links== | |||
==References== | ==References== | ||
| Line 49: | Line 63: | ||
<references/> | <references/> | ||
[[Category:Radiology]] | [[Category:Radiology]] | ||
[[Category:Renal]] | |||
Latest revision as of 17:23, 22 March 2023
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
Clinical Features
- 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 induced complications
- Contrast induced allergic reaction
- Contrast-induced nephropathy
- CT contrast media extravasation
- Nephrogenic systemic fibrosis
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
External Links
References
- Scheinfeld NS et Al. Nephrogenic systemic fibrosis. eMedicine. Updated Feb 8, 2016. http://emedicine.medscape.com/article/1097889-overview.