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nephrogenic systemic fibrosis, imatinib, corticosteroids, treatment
Nephrogenic systemic fibrosis (NSF) is a sclerotic disorder presenting with painful indurated plaques and skin thickening involving the trunk and extremities, which can lead to tethering and joint contractures. NSF most commonly affects patients with renal insufficiency who have been exposed to gadolinium. We present a case of NSF involving the bilateral hands, knees, and lower extremities developing over 10 years after gadolinium exposure. Initial improvement was noted in the lower extremities after initiation of imatinib mesylate therapy, but recalcitrant, thickened hand plaques caused persistent pain and functional limitation. Adjunct intralesional corticosteroid injections produced durable softening of the recalcitrant lesions with considerable functional improvement in hand mobility. Based on our experience, intralesional corticosteroid injections appear to be an effective adjunct treatment in patients with incomplete response to anti-fibrotic therapies.
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