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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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| ==Historical Background== | | ==[[Nephrogenic fibrosing dermopathy historical perspective|Historical Perspective]]== |
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| The first cases of NFD have been identified in 1997 as a fibrotic disorder of the skin in patients with renal failure .<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> Since then, systemic involvement has been described in some patients with NFD, resulting in use of the term nephrogenic systemic fibrosis (NSF); NFD and NSF have been used to describe the same condition (3). No clear etiology has been established for NFD, and little is known about its pathogenesis or natural history. This report describes the largest geographic cluster of NFD that has been identified and provides evidence that exposure to a gadolinium-containing contrast agent is a risk factor for the development of the disease. 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>
| | ==[[Nephrogenic fibrosing dermopathy classification scheme|Classification]]== |
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| 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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| Although risk factors for NFD have not been studied extensively, possible correlations with severity of renal failure, thrombotic episodes, edema, and vascular procedures have been reported (2,4). Recently, medication exposures such as erythropoietin and gadolinium-containing contrast agents have been identified as potential risk factors for NFD (5,6). In May 2006, the Danish Medicines Agency reported 25 cases of NFD diagnosed in Europe among patients with recent exposure to gadolinium-containing contrast. In response, the Food and Drug Administration (FDA) issued a public health advisory in June 2006 regarding the use of these contrast agents in patients with renal failure (7). As of December 25, 2006, the FDA MedWatch system had received 90 reports of NFD possibly related to gadolinium-containing contrast agents.
| | ==[[Nephrogenic fibrosing dermopathy causes|Causes]]== |
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| Intravenously administered contrast agents are used routinely for MRI studies; the contrast agents contain gadolinium (a paramagnetic heavy metal), which is bound to a chelating agent. The mechanism for possible gadolinium-associated NFD is unknown; however, one hypothesis is that the gadolinium ions might dissociate from the chelate and result in a fibrotic reaction (5). Five gadolinium-based contrast agents are available in the United States; the first was approved for use in 1988 (7). Adverse events associated with these agents typically are minor (e.g., nausea); severe effects such as allergic reactions or tissue necrosis as a result of extravasation are rare. In addition, gadolinium-containing contrast agents are believed to be less nephrotoxic than iodinated contrast agents used for computed tomography (CT) imaging (8). Excretion of gadolinium-containing contrast agents primarily occurs renally; the amount of contrast eliminated from the body after dialysis has not been well-evaluated. Two studies suggest that 65%--78% of gadolinium-containing contrast might be cleared after one hemodialysis session and 98% after three sessions (9,10). Peritoneal dialysis might achieve less effective gadolinium-contrast clearance than hemodialysis. In one study, 69% of total gadolinium-containing contrast was excreted after 22 days in patients undergoing continuous ambulatory peritoneal dialysis (9). Delayed clearance might prolong the duration of gadolinium-containing contrast exposure among patients undergoing peritoneal dialysis. However, patients undergoing peritoneal dialysis have not been previously reported to be at higher risk for NFD than patients undergoing hemodialysis. The chronic peritoneal dialysis outpatients in this investigation had higher estimated NFD attack rates than chronic hemodialysis outpatients. No controls who had gadolinium-containing contrast exposure underwent primarily peritoneal dialysis.
| | ==[[Nephrogenic fibrosing dermopathy differential diagnosis|Differentiating Nephrogenic fibrosing dermopathy from other Diseases]]== |
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| ==Clinical Presentations== | | ==[[Nephrogenic fibrosing dermopathy epidemiology and demographics|Epidemiology and Demographics]]== |
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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 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== | | ==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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| 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]].
| | ==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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| ==Classification==
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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>
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| *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>
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| ==References==
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| {{reflist|2}}
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| [[de:Nephrogene systemische Fibrose]] | | [[de:Nephrogene systemische Fibrose]] |
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| [[Category:Diseases]] | | [[Category:Disease]] |
| [[Category:Dermatology]] | | [[Category:Dermatology]] |
| [[Category:Nephrology]] | | [[Category:Nephrology]] |
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