TY - JOUR
T1 - Nephrogenic Systemic Fibrosis
T2 - Clinical Picture and Treatment
AU - Marckmann, Peter
AU - Skov, Lone
PY - 2009/9/1
Y1 - 2009/9/1
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.
KW - Chronic kidney disease
KW - Clinical picture
KW - Diagnosis
KW - Gadolinium
KW - Nephrogenic systemic fibrosis
KW - Renal failure
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=69649095947&partnerID=8YFLogxK
U2 - 10.1016/j.rcl.2009.05.004
DO - 10.1016/j.rcl.2009.05.004
M3 - Review
C2 - 19744598
AN - SCOPUS:69649095947
VL - 47
SP - 833
EP - 840
JO - Radiologic Clinics of North America
JF - Radiologic Clinics of North America
SN - 0033-8389
IS - 5
ER -