NEPHROGENIC SYSTEMIC FIBROSIS Ahmed Donia, MD, MRCP (UK)

DEFINITION Nephrogenic systemic fibrosis (NSF) is a recently identified fibrosing disorder seen only in patients with kidney failure.

ETIOLOGY Gadolinium usage especially in high doses in renal failure patients with GFR less than 30 ml/minute (some reports in patients with GFR ml/min) Remember that GFR will be less than 30 e.g. in a 60 year-old woman weighing 60 kg with a creatinine of only 2 mg/dl (GRF can be calculated using Cockcroft- Gault or MDRD formulas) Remember that MRI can be done without contrast….in such case there is no problem

Clinical manifestations –Latent period: 2-4 weeks –2-5% of susceptible patients –Skin involvement Skin disease in NSF typically presents as symmetrical, bilateral fibrotic indurated papules, plaques, or subcutaneous nodules that may or may not be erythematous In the majority of cases, the lesions first develop on the ankles, lower legs, feet, and hands and then move proximally to involve the thighs, forearms, and, less often, the trunk or buttocks. The lesions are often preceded by edema and may initially be misdiagnosed as cellulitis –Systemic involvement — The prevalence of systemic involvement is unknown, but a number of different organ system manifestations have been described

Diagnosis –History –Examination –Skin biopsy –Systemic markers of inflammation

Prevention Avoidance of gadolinium in the susceptible patients In susceptible patients, the usage of CT with contrast and following the protocol for contrast nephropathy prevention will be better If gadolinium must be used –The patient must be fully understanding –Use a relatively safer gadolinium preparation….the worst is omniscan (gadodiamide) –The least possible dose –Post-contrast dialysis session (remember that a dialysis after CT with contrast is useless)

Treatment NO PROVEN THERAPY –Kidney transplantation –Extracorporeal photopheresis –Plasmapherisis –UV A phototherapy