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Nephrogenic systemic fibrosis

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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 MRIs) in patients with severe kidney failure. It does not have a genetic basis.

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 fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin. [1]

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 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 OptiMARK.

As a condition, NFD was first observed in 1997. As an independent disease entity, it was first described in 2000.[2] While skin involvement is on the foreground, the process may involve any organ and resembles diffuse scleroderma or systemic sclerosis. [3] In 2006, the link between NFD and gadolinium-containing contrast agents was made.[4][5][6] 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.[7]

The European authorities have classified the gadolinium-containing contrast agents in three groups:[8]

  • Least likely (safest) to release free gadolinium ions Gd3+ in the body have a cyclical structure: Dotarem, Gadovist and ProHance
  • Intermediate have a ionic linear structure: Magnevist, MultiHance, Primovist and Vasovist
  • Most likely to release Gd3+ have a linear non-ionic structure: Omniscan and OptiMARK

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).[9]

References

  1. ^ Scheinfeld, NS; Cowper, S; Kovarik, CL; Butler, DF. "Nephrogenic Fibrosing Dermatopathy." Emedicine. [1]
  2. ^ Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE (2000). "Scleromyxoedema-like cutaneous diseases in renal-dialysis patients". Lancet. 356 (9234): 1000–1. PMID 11041404.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  3. ^ Mendoza FA, Artlett CM, Sandorfi N, Latinis K, Piera-Velazquez S, Jimenez SA (2006). "Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature". Semin. Arthritis Rheum. 35 (4): 238–49. doi:10.1016/j.semarthrit.2005.08.002. PMID 16461069.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  4. ^ Grobner T (2006). "Gadolinium--a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis?". Nephrol. Dial. Transplant. 21 (4): 1104–8. doi:10.1093/ndt/gfk062. PMID 16431890.
  5. ^ Marckmann P, Skov L, Rossen K; et al. (2006). "Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging". J. Am. Soc. Nephrol. 17 (9): 2359–62. doi:10.1681/ASN.2006060601. PMID 16885403. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
  6. ^ "Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents--St. Louis, Missouri, 2002-2006". MMWR Morb. Mortal. Wkly. Rep. 56 (7): 137–41. 2007. PMID 17318112.
  7. ^ Kanal E, Barkovich AJ, Bell C; et al. (2007). "ACR guidance document for safe MR practices: 2007". AJR. American journal of roentgenology. 188 (6): 1447–74. doi:10.2214/AJR.06.1616. PMID 17515363. {{cite journal}}: Explicit use of et al. in: |author= (help)CS1 maint: multiple names: authors list (link)
  8. ^ http://www.mhra.gov.uk/home/idcplg?IdcService=GET_FILE&dID=35149&noSaveAs=0&Rendition=WEB
  9. ^ http://www.ismrm.org/special/EMEA2.pdf
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