TY - JOUR
T1 - Nephrogenic systemic fibrosis: clinical picture and treatment
AU - Marckmann, Peter
AU - Skov, Lone
N1 - Keywords: Contrast Media; Diagnosis, Differential; Gadolinium; Humans; Kidney Function Tests; Nephrogenic Fibrosing Dermopathy; Risk Factors
PY - 2009
Y1 - 2009
N2 - 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.
AB - 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.
U2 - 10.1016/j.rcl.2009.05.004
DO - 10.1016/j.rcl.2009.05.004
M3 - Journal article
C2 - 19744598
SN - 0033-8389
VL - 47
SP - 833-40, vi
JO - Radiologic Clinics of North America
JF - Radiologic Clinics of North America
IS - 5
ER -