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Nephrogenic systemic fibrosis

MedGen UID:
854737
Concept ID:
C3888044
Disease or Syndrome
Synonym: Nephrogenic Fibrosing Dermopathy
SNOMED CT: Nephrogenic systemic fibrosis (424114000)
 
Monarch Initiative: MONDO:0015294
Orphanet: ORPHA137617

Definition

Nephrogenic systemic fibrosis (NSF) is a rare systemic fibrosing condition observed in renally impaired patients and characterized by a hardening and thickening of the skin with fibrotic plaques or papules, pruritus, joint pain and stiffness, muscle weakness, limitation of range of motion, and yellowed eyes. It is generally associated with administration of gadolinium-based magnetic resonance imaging contrast agents (GBCA) in patients with kidney disease. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNephrogenic systemic fibrosis

Professional guidelines

PubMed

Weinreb JC, Rodby RA, Yee J, Wang CL, Fine D, McDonald RJ, Perazella MA, Dillman JR, Davenport MS
Radiology 2021 Jan;298(1):28-35. Epub 2020 Nov 10 doi: 10.1148/radiol.2020202903. PMID: 33170103
Orteu CH, Ong VH, Denton CP
Best Pract Res Clin Rheumatol 2020 Feb;34(1):101489. Epub 2020 Mar 5 doi: 10.1016/j.berh.2020.101489. PMID: 32147386
Thomsen HS; European Society of Urogenital Radiology (ESUR)
Eur Radiol 2007 Oct;17(10):2692-6. doi: 10.1007/s00330-007-0744-5. PMID: 17977076

Recent clinical studies

Etiology

Choi JW, Moon WJ
Korean J Radiol 2019 Jan;20(1):134-147. Epub 2018 Dec 27 doi: 10.3348/kjr.2018.0356. PMID: 30627029Free PMC Article
Shah AH, Olivero JJ
Methodist Debakey Cardiovasc J 2017 Jul-Sep;13(3):172-173. doi: 10.14797/mdcj-13-3-172. PMID: 29744004Free PMC Article
Zamora CA, Castillo M
Magn Reson Imaging Clin N Am 2017 Nov;25(4):685-696. Epub 2017 Aug 23 doi: 10.1016/j.mric.2017.06.002. PMID: 28964458
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039
Kaewlai R, Abujudeh H
AJR Am J Roentgenol 2012 Jul;199(1):W17-23. doi: 10.2214/AJR.11.8144. PMID: 22733927

Diagnosis

Leisman S
Adv Chronic Kidney Dis 2020 Jan;27(1):50-55. doi: 10.1053/j.ackd.2019.08.002. PMID: 32147002
Rongioletti F, Ferreli C, Atzori L, Bottoni U, Soda G
G Ital Dermatol Venereol 2018 Apr;153(2):208-215. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05922-9. PMID: 29368844
Shah AH, Olivero JJ
Methodist Debakey Cardiovasc J 2017 Jul-Sep;13(3):172-173. doi: 10.14797/mdcj-13-3-172. PMID: 29744004Free PMC Article
Zamora CA, Castillo M
Magn Reson Imaging Clin N Am 2017 Nov;25(4):685-696. Epub 2017 Aug 23 doi: 10.1016/j.mric.2017.06.002. PMID: 28964458
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039

Therapy

Cheong BYC, Wilson JM, Preventza OA, Muthupillai R
Tex Heart Inst J 2022 May 1;49(3) doi: 10.14503/THIJ-21-7680. PMID: 35612906Free PMC Article
Leisman S
Adv Chronic Kidney Dis 2020 Jan;27(1):50-55. doi: 10.1053/j.ackd.2019.08.002. PMID: 32147002
Choi JW, Moon WJ
Korean J Radiol 2019 Jan;20(1):134-147. Epub 2018 Dec 27 doi: 10.3348/kjr.2018.0356. PMID: 30627029Free PMC Article
Shah AH, Olivero JJ
Methodist Debakey Cardiovasc J 2017 Jul-Sep;13(3):172-173. doi: 10.14797/mdcj-13-3-172. PMID: 29744004Free PMC Article
Leiner T, Kucharczyk W
J Magn Reson Imaging 2009 Dec;30(6):1233-5. doi: 10.1002/jmri.21985. PMID: 19938034

Prognosis

Lyerly MJ, Chow D
J Stroke Cerebrovasc Dis 2021 Sep;30(9):105930. Epub 2021 Jun 24 doi: 10.1016/j.jstrokecerebrovasdis.2021.105930. PMID: 34176719
Shah AH, Olivero JJ
Methodist Debakey Cardiovasc J 2017 Jul-Sep;13(3):172-173. doi: 10.14797/mdcj-13-3-172. PMID: 29744004Free PMC Article
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039
Kooman JP
J Magn Reson Imaging 2009 Dec;30(6):1341-6. doi: 10.1002/jmri.21970. PMID: 19937932
Rosenkranz AR, Grobner T, Mayer GJ
Wien Klin Wochenschr 2007;119(9-10):271-5. doi: 10.1007/s00508-007-0801-8. PMID: 17571229

Clinical prediction guides

Lyerly MJ, Chow D
J Stroke Cerebrovasc Dis 2021 Sep;30(9):105930. Epub 2021 Jun 24 doi: 10.1016/j.jstrokecerebrovasdis.2021.105930. PMID: 34176719
Alfano G, Fontana F, Ferrari A, Solazzo A, Perrone R, Giaroni F, Torricelli P, Cappelli G
Magn Reson Imaging 2020 Jul;70:1-4. Epub 2020 Feb 26 doi: 10.1016/j.mri.2020.02.012. PMID: 32112811
Tsushima Y, Awai K, Shinoda G, Miyoshi H, Chosa M, Sunaya T, Endrikat J
Jpn J Radiol 2018 Nov;36(11):676-685. Epub 2018 Sep 19 doi: 10.1007/s11604-018-0778-4. PMID: 30232584
Garcia J, Liu SZ, Louie AY
Philos Trans A Math Phys Eng Sci 2017 Nov 28;375(2107) doi: 10.1098/rsta.2017.0180. PMID: 29038383Free PMC Article
Martin DR
Pediatr Radiol 2008 Jan;38 Suppl 1:S125-9. Epub 2007 Dec 11 doi: 10.1007/s00247-007-0589-8. PMID: 18071694

Recent systematic reviews

Shankar PR, Davenport MS
Radiology 2020 Nov;297(2):447-448. Epub 2020 Aug 18 doi: 10.1148/radiol.2020201492. PMID: 32808890
Lunyera J, Mohottige D, Alexopoulos AS, Campbell H, Cameron CB, Sagalla N, Amrhein TJ, Crowley MJ, Dietch JR, Gordon AM, Kosinski AS, Cantrell S, Williams JW Jr, Gierisch JM, Ear B, Goldstein KM
Ann Intern Med 2020 Jul 21;173(2):110-119. Epub 2020 Jun 23 doi: 10.7326/M20-0299. PMID: 32568573Free PMC Article
Woolen SA, Shankar PR, Gagnier JJ, MacEachern MP, Singer L, Davenport MS
JAMA Intern Med 2020 Feb 1;180(2):223-230. doi: 10.1001/jamainternmed.2019.5284. PMID: 31816007Free PMC Article
Attari H, Cao Y, Elmholdt TR, Zhao Y, Prince MR
Radiology 2019 Aug;292(2):376-386. Epub 2019 Jul 2 doi: 10.1148/radiol.2019182916. PMID: 31264946
Agarwal R, Brunelli SM, Williams K, Mitchell MD, Feldman HI, Umscheid CA
Nephrol Dial Transplant 2009 Mar;24(3):856-63. Epub 2008 Oct 24 doi: 10.1093/ndt/gfn593. PMID: 18952698

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