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Nephrogenic systemic fibrosis
A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance i...
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| Published in: | Radiologe 2007-09, Vol.47 (9), p.778 |
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| Format: | Article |
| Language: | ger |
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| container_title | Radiologe |
| container_volume | 47 |
| creator | Samtleben, W |
| description | A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) |
| doi_str_mv | 10.1007/s00117-007-1545-1 |
| format | article |
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In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) <30 ml/min per 1.73 m(2). So far a total of about 215 patients with this new skin disorder have been reported to international registries. The skin thickening has a typical histology and begins in the peripheral extremities and progresses proximally, including also the abdominal wall and the head in some patients. NSF involves not only the skin, but also the muscles and other organs (e.g., lungs, heart, eyes) in some patients. Therefore the term nephrogenic systemic fibrosis (NSF) was introduced. Skin fibrosis and sclerosis are usually progressive with disabling contractures of involved joints (knees, hands, feet). NSF may be lethal in up to 28% of patients. Spontaneous remissions are rare. No generally accepted treatment is available. So far, the pathogenesis is not well understood. One hypothesis supposes a role of gadolinium liberated from the contrast agents. As patients with acute or chronic advanced renal failure (GFR <30 ml/min per 1.73 m(2)) including those with hepatorenal dysfunctions are at high risk to develop NSF after exposure to gadolinium-based contrast agents, contrast-enhanced MRI should be avoided in this group and alternative diagnostic procedures should be used whenever possible.</description><identifier>ISSN: 0033-832X</identifier><identifier>DOI: 10.1007/s00117-007-1545-1</identifier><identifier>PMID: 17717643</identifier><language>ger</language><publisher>Germany</publisher><subject>Contrast Media - adverse effects ; Disease Progression ; Fibrosis - chemically induced ; Fibrosis - pathology ; Gadolinium DTPA - adverse effects ; Glomerular Filtration Rate ; Humans ; Kidney Transplantation ; Magnetic Resonance Imaging - adverse effects ; Male ; Middle Aged ; Renal Dialysis ; Renal Insufficiency - complications ; Renal Insufficiency - therapy ; Risk Factors ; Scleromyxedema - chemically induced ; Skin - pathology ; Skin Diseases - chemically induced ; Skin Diseases - pathology</subject><ispartof>Radiologe, 2007-09, Vol.47 (9), p.778</ispartof><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,777,781,27905,27906</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17717643$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Samtleben, W</creatorcontrib><title>Nephrogenic systemic fibrosis</title><title>Radiologe</title><addtitle>Radiologe</addtitle><description>A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) <30 ml/min per 1.73 m(2). So far a total of about 215 patients with this new skin disorder have been reported to international registries. The skin thickening has a typical histology and begins in the peripheral extremities and progresses proximally, including also the abdominal wall and the head in some patients. NSF involves not only the skin, but also the muscles and other organs (e.g., lungs, heart, eyes) in some patients. Therefore the term nephrogenic systemic fibrosis (NSF) was introduced. Skin fibrosis and sclerosis are usually progressive with disabling contractures of involved joints (knees, hands, feet). NSF may be lethal in up to 28% of patients. Spontaneous remissions are rare. No generally accepted treatment is available. So far, the pathogenesis is not well understood. One hypothesis supposes a role of gadolinium liberated from the contrast agents. As patients with acute or chronic advanced renal failure (GFR <30 ml/min per 1.73 m(2)) including those with hepatorenal dysfunctions are at high risk to develop NSF after exposure to gadolinium-based contrast agents, contrast-enhanced MRI should be avoided in this group and alternative diagnostic procedures should be used whenever possible.</description><subject>Contrast Media - adverse effects</subject><subject>Disease Progression</subject><subject>Fibrosis - chemically induced</subject><subject>Fibrosis - pathology</subject><subject>Gadolinium DTPA - adverse effects</subject><subject>Glomerular Filtration Rate</subject><subject>Humans</subject><subject>Kidney Transplantation</subject><subject>Magnetic Resonance Imaging - adverse effects</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Renal Dialysis</subject><subject>Renal Insufficiency - complications</subject><subject>Renal Insufficiency - therapy</subject><subject>Risk Factors</subject><subject>Scleromyxedema - chemically induced</subject><subject>Skin - pathology</subject><subject>Skin Diseases - chemically induced</subject><subject>Skin Diseases - pathology</subject><issn>0033-832X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><recordid>eNo1js1KAzEURu9CsbX2AVwovkD03tz8LqVoFYpuFLorySRpRxw7TOqib--Aujpncfj4AC4JbwnR3lVEIitGFaSVFnQCU0Rm4ViuJ3Be68dYSC_9GUzIWrJG8RSuXnK_G_bb_NU2N_VYD7kbpbRx2Ne2XsBpCZ81z_84g_fHh7fFk1i9Lp8X9yvRE7qDkLqkgNlJHXx0OqGR6JNK1rjGU9QGDWqvrJPcROOjVwFDdE1hW9gQ8gyuf3f779jltOmHtgvDcfP_k38Aqk48HQ</recordid><startdate>200709</startdate><enddate>200709</enddate><creator>Samtleben, W</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope></search><sort><creationdate>200709</creationdate><title>Nephrogenic systemic fibrosis</title><author>Samtleben, W</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p108t-25fda0e825a9b85d06209d4d768c91b560605947823cb69b94a0ab8cf37f36103</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>ger</language><creationdate>2007</creationdate><topic>Contrast Media - adverse effects</topic><topic>Disease Progression</topic><topic>Fibrosis - chemically induced</topic><topic>Fibrosis - pathology</topic><topic>Gadolinium DTPA - adverse effects</topic><topic>Glomerular Filtration Rate</topic><topic>Humans</topic><topic>Kidney Transplantation</topic><topic>Magnetic Resonance Imaging - adverse effects</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Renal Dialysis</topic><topic>Renal Insufficiency - complications</topic><topic>Renal Insufficiency - therapy</topic><topic>Risk Factors</topic><topic>Scleromyxedema - chemically induced</topic><topic>Skin - pathology</topic><topic>Skin Diseases - chemically induced</topic><topic>Skin Diseases - pathology</topic><toplevel>online_resources</toplevel><creatorcontrib>Samtleben, W</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><jtitle>Radiologe</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Samtleben, W</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Nephrogenic systemic fibrosis</atitle><jtitle>Radiologe</jtitle><addtitle>Radiologe</addtitle><date>2007-09</date><risdate>2007</risdate><volume>47</volume><issue>9</issue><spage>778</spage><pages>778-</pages><issn>0033-832X</issn><abstract>A scleromyxedema-like disease was recognized in 1997. In 2000 this disorder was first published and termed nephrogenic fibrosing dermopathy because all patients had advanced renal failure. In 2006 it was discovered that the patients had a history of a preceding contrast-enhanced magnetic resonance imaging (MRI). All patients had acute or chronic severe renal insufficiency with a glomerular filtration rate (GFR) <30 ml/min per 1.73 m(2). So far a total of about 215 patients with this new skin disorder have been reported to international registries. The skin thickening has a typical histology and begins in the peripheral extremities and progresses proximally, including also the abdominal wall and the head in some patients. NSF involves not only the skin, but also the muscles and other organs (e.g., lungs, heart, eyes) in some patients. Therefore the term nephrogenic systemic fibrosis (NSF) was introduced. Skin fibrosis and sclerosis are usually progressive with disabling contractures of involved joints (knees, hands, feet). NSF may be lethal in up to 28% of patients. Spontaneous remissions are rare. No generally accepted treatment is available. So far, the pathogenesis is not well understood. One hypothesis supposes a role of gadolinium liberated from the contrast agents. As patients with acute or chronic advanced renal failure (GFR <30 ml/min per 1.73 m(2)) including those with hepatorenal dysfunctions are at high risk to develop NSF after exposure to gadolinium-based contrast agents, contrast-enhanced MRI should be avoided in this group and alternative diagnostic procedures should be used whenever possible.</abstract><cop>Germany</cop><pmid>17717643</pmid><doi>10.1007/s00117-007-1545-1</doi></addata></record> |
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| identifier | ISSN: 0033-832X |
| ispartof | Radiologe, 2007-09, Vol.47 (9), p.778 |
| issn | 0033-832X |
| language | ger |
| recordid | cdi_pubmed_primary_17717643 |
| source | Alma/SFX Local Collection |
| subjects | Contrast Media - adverse effects Disease Progression Fibrosis - chemically induced Fibrosis - pathology Gadolinium DTPA - adverse effects Glomerular Filtration Rate Humans Kidney Transplantation Magnetic Resonance Imaging - adverse effects Male Middle Aged Renal Dialysis Renal Insufficiency - complications Renal Insufficiency - therapy Risk Factors Scleromyxedema - chemically induced Skin - pathology Skin Diseases - chemically induced Skin Diseases - pathology |
| title | Nephrogenic systemic fibrosis |
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