Nephrogenic systemic fibrosis: clinical picture and treatment

Peter Marckmann; Lone Skov

doi:10.1016/j.rcl.2009.05.004

Nephrogenic systemic fibrosis: clinical picture and treatment

Peter Marckmann, Lone Skov

27 Citations (Scopus)

Abstract

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.

Original language	English
Journal	Radiologic Clinics of North America
Volume	47
Issue number	5
Pages (from-to)	833-40, vi
Number of pages	8
ISSN	0033-8389
DOIs	https://doi.org/10.1016/j.rcl.2009.05.004
Publication status	Published - 2009

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10.1016/j.rcl.2009.05.004

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title = "Nephrogenic systemic fibrosis: clinical picture and treatment",

abstract = "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.",

author = "Peter Marckmann and Lone Skov",

note = "Keywords: Contrast Media; Diagnosis, Differential; Gadolinium; Humans; Kidney Function Tests; Nephrogenic Fibrosing Dermopathy; Risk Factors",

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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 -

Nephrogenic systemic fibrosis: clinical picture and treatment

Abstract

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