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| {{SI}} | | __NOTOC__ |
| | {{Nephrogenic fibrosing dermopathy}} |
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| {{CMG}} | | {{CMG}}''' Assistant Editor-in-Chief:''' [[Brian Blank]] |
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| {{EJ}} | | {{SK}} nephrogenic systemic fibrosis |
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| ==Overview== | | ==[[Nephrogenic fibrosing dermopathy overview|Overview]]== |
| '''Nephrogenic fibrosing dermopathy''' or '''nephrogenic systemic fibrosis''' is a rare and serious [[syndrome]] that involves [[fibrosis]] of skin, joints, eyes, and internal organs. Its cause is not fully understood, but it seems to be associated with exposure to [[gadolinium]] (which is frequently used as a contrast substance for [[MRI]]s) in patients with severe [[renal failure|kidney failure]]. It does not have a genetic basis.
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| In NFD, patients develop large areas of hardened skin with fibrotic nodules and plaques. Flexion contractures with an accompanying limitation of range of motion can also occur. NFD resembles scleromyxedema at the histologic (microscopic) level; it shows a proliferation of dermal [[fibroblast]]s and [[dendritic cells]], thickened [[collagen]] bundles, increased [[elastic fiber]]s, and deposits of [[mucin]]. <ref> Scheinfeld, NS; Cowper, S; Kovarik, CL; Butler, DF. "Nephrogenic Fibrosing Dermatopathy." ''Emedicine.'' [http://www.emedicine.com/DERM/topic934.htm] </ref>
| | ==[[Nephrogenic fibrosing dermopathy historical perspective|Historical Perspective]]== |
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| Most patients with NFD have undergone [[hemodialysis]] for renal failure, some have never undergone dialysis and others have received only [[peritoneal dialysis]]. Many patients have taken [[immunosuppressant|immunosuppressive]] medications and have other diseases, such as [[hepatitis C]]. Four of the five [[FDA]]-approved gadolinium contrast agents have been principally implicated in NFD, including Omniscan, Multihance, Magnevist, and [[Gadoversetamide|OptiMARK]].
| | ==[[Nephrogenic fibrosing dermopathy classification scheme|Classification]]== |
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| The first cases of NFD have been identified in 1997.<ref>{{cite journal |author=Cowper SE |title=Nephrogenic fibrosing dermopathy: the first 6 years |journal=Current Opinion in Rheumatology |volume=15 |pages=785-790 |year=2003}}</ref>As an independent disease entity, NFD was first described in 2000.<ref>{{cite journal |author=Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE |title=Scleromyxoedema-like cutaneous diseases in renal-dialysis patients |journal=Lancet |volume=356 |issue=9234 |pages=1000-1 |year=2000 |pmid=11041404 |doi=}}</ref> While skin involvement is on the foreground, the process may involve any organ and resembles diffuse scleroderma or systemic sclerosis. <ref>{{cite journal |author=Mendoza FA, Artlett CM, Sandorfi N, Latinis K, Piera-Velazquez S, Jimenez SA |title=Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature |journal=Semin. Arthritis Rheum. |volume=35 |issue=4 |pages=238-49 |year=2006 |pmid=16461069 |doi=10.1016/j.semarthrit.2005.08.002}}</ref> In 2006, the link between NFD and gadolinium-containing contrast agents was made.<ref>{{cite journal |author=Grobner T |title=Gadolinium--a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? |journal=Nephrol. Dial. Transplant. |volume=21 |issue=4 |pages=1104-8 |year=2006 |pmid=16431890 |doi=10.1093/ndt/gfk062}}</ref><ref>{{cite journal |author=Marckmann P, Skov L, Rossen K, ''et al'' |title=Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging |journal=J. Am. Soc. Nephrol. |volume=17 |issue=9 |pages=2359-62 |year=2006 |pmid=16885403 |doi=10.1681/ASN.2006060601}}</ref><ref>{{cite journal |author= |title=Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents--St. Louis, Missouri, 2002-2006 |journal=MMWR Morb. Mortal. Wkly. Rep. |volume=56 |issue=7 |pages=137-41 |year=2007 |pmid=17318112 |doi=}}</ref> As a result, gadolinium-containing contrast is now considered contraindicated in patients with an estimated [[glomerular filtration rate]] (a measure of [[renal function]]) under 60 and especially under 30.<ref>{{cite journal |author=Kanal E, Barkovich AJ, Bell C, ''et al'' |title=ACR guidance document for safe MR practices: 2007 |journal=AJR. American journal of roentgenology |volume=188 |issue=6 |pages=1447-74 |year=2007 |pmid=17515363 |doi=10.2214/AJR.06.1616}}</ref>
| | ==[[Nephrogenic fibrosing dermopathy pathophysiology|Pathophysiology]]== |
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| The European authorities have classified the gadolinium-containing contrast agents in three groups:<ref>http://www.mhra.gov.uk/home/idcplg?IdcService=GET_FILE&dID=35149&noSaveAs=0&Rendition=WEB</ref>
| | ==[[Nephrogenic fibrosing dermopathy causes|Causes]]== |
| *Least likely (safest) to release free gadolinium ions Gd3+ in the body have a cyclical structure: Dotarem, Gadovist and ProHance
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| *Intermediate have a ionic linear structure: Magnevist, MultiHance, Primovist and Vasovist
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| *Most likely to release Gd3+ have a linear non-ionic structure: Omniscan and OptiMARK
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| It can be noted that this classification was released after another proposition would have left the safest category (ionic cyclical structure) with only one agent (Dotarem). The intermediate category would have been either ionic linear structure or non-ionic cyclical structure. The third category most at risk was unchanged (linear non-ionic).<ref>http://www.ismrm.org/special/EMEA2.pdf</ref>
| | ==[[Nephrogenic fibrosing dermopathy differential diagnosis|Differentiating Nephrogenic fibrosing dermopathy from other Diseases]]== |
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| | ==[[Nephrogenic fibrosing dermopathy epidemiology and demographics|Epidemiology and Demographics]]== |
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| | ==[[Nephrogenic fibrosing dermopathy risk factors|Risk Factors]]== |
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| | ==[[Nephrogenic fibrosing dermopathy natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| | ==Diagnosis== |
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| | [[Nephrogenic fibrosing dermopathy history and symptoms|History and Symptoms ]] | [[ Nephrogenic fibrosing dermopathy physical examination|Physical Examination]] | [[Nephrogenic fibrosing dermopathy laboratory findings|Laboratory Findings]] | [[Nephrogenic fibrosing dermopathy x ray|X-Ray]] | [[Nephrogenic fibrosing dermopathy CT|CT]] | [[Nephrogenic fibrosing dermopathy MRI|MRI]] | [[Nephrogenic fibrosing dermopathy ultrasound|Ultrasound]] | [[Nephrogenic fibrosing dermopathy other imaging findings|Other Imaging Findings]] | [[Nephrogenic fibrosing dermopathy other diagnostic studies|Other Diagnostic Studies]] |
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| | ==Treatment== |
| | [[Nephrogenic fibrosing dermopathy medical therapy|Medical Therapy]] | [[Nephrogenic fibrosing dermopathy surgery |Surgery]] | [[Nephrogenic fibrosing dermopathy secondary prevention|Secondary Prevention]] | [[Nephrogenic fibrosing dermopathy cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Nephrogenic fibrosing dermopathy future or investigational therapies|Future or Investigational Therapies]] |
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| | ==Case Studies== |
| | [[Nephrogenic fibrosing dermopathy case study one|Case #1]] |
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| | ==Source== |
| | * [http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5607a1.htm CDC] |
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| ==References==
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| {{reflist|2}}
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| [[de:Nephrogene systemische Fibrose]]
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| [[Category:Diseases]]
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| [[Category:Dermatology]]
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| [[Category:Nephrology]]
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| {{SIB}}
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| | [[de:Nephrogene systemische Fibrose]] |
| {{WH}} | | {{WH}} |
| {{WS}} | | {{WS}} |
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| | [[Category:Disease]] |
| | [[Category:Dermatology]] |
| | [[Category:Nephrology]] |