Nephrogenic systemic fibrosis

Published on 18/03/2015 by admin

Filed under Dermatology

Last modified 18/03/2015

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Nephrogenic systemic fibrosis

Anjela Galan and Shawn E. Cowper

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

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(From Nazarian, R.M., et al., 2011. Quantitative assessment of dermal cellularity in nephrogenic systemic fibrosis: a diagnostic aid. J Am Acad Dermatol 64(4), 741–7.)

Nephrogenic systemic fibrosis (NSF) affects patients with renal impairment who have been exposed to gadolinium-containing contrast agents (GCCA) for MRI studies. Onset is marked by cutaneous erythema, edema, hyperpigmentation, and woody induration. The clinical course commonly results in joint contractures and sometimes fibrosis of internal organs.

Management strategy

Prevention is paramount. Three GCCA (Magnevist, Omniscan, and Optimark) are now contraindicated in patients with renal dysfunction (acute kidney injury or chronic kidney disease [estimated GFR of <30 mL/minute/1.73 m2]). Patients should be screened prior to the use of GCCA to identify those at risk. Avoid using GCCA in patients with suspected or known impairment of drug elimination unless the need for the diagnostic information is essential and not available with non-contrasted MRI or other alternative imaging modalities. If exposure is unavoidable, optimal dosing and follow-up should involve the managing radiologist and nephrologist. Although not proven to prevent NSF, immediate hemodialysis following exposure to GCCA is recommended.

Specific investigations

There are no serologic tests for NSF. Diagnosis is based upon clinical and histopathological correlation in a patient with renal disease. Gadolinium identification is not required to establish a diagnosis.

First-line therapies

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