Nephrogenic systemic fibrosis: clinical picture and treatment

The classic hallmark symptoms of advanced nephrogenic systemic fibrosis (NSF) (skin thickening, hardening and hyperpigmentation, and disabling contractures in renal failure patients) in temporal association with Gd-based contrast agent (GBCA) exposure are almost pathognomonic of NSF. Less obvious cases may be diagnosed on the basis of history of early GBCA-related NSF symptoms (warm swellings, pain, discoloration, itching of lower legs), signs of multiorgan involvement (lungs, nervous system), the exclusion of differential diagnoses, including scleromyxedema and systemic sclerosis, and the histology of deep skin biopsies. Symptomatic treatment with intensive physiotherapy and painkillers is important, but there is no known curative medical treatment. Spontaneous remission of NSF symptoms may occur with recovery of renal function after an episode of acute renal failure, or with kidney transplantation of chronic renal failure patients.