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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
Fraum TJ, Ludwig DR, Bashir MR, Fowler KJ
J Magn Reson Imaging 2017 Aug;46(2):338-353. Epub 2017 Jan 13 doi: 10.1002/jmri.25625. PMID: 28083913

Recent clinical studies

Etiology

Starekova J, Pirasteh A, Reeder SB
AJR Am J Roentgenol 2024 Sep;223(3):e2330036. Epub 2023 Nov 15 doi: 10.2214/AJR.23.30036. PMID: 37850581
Leisman S
Adv Chronic Kidney Dis 2020 Jan;27(1):50-55. doi: 10.1053/j.ackd.2019.08.002. PMID: 32147002
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
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
Todd DJ, Kay J
Annu Rev Med 2016;67:273-91. doi: 10.1146/annurev-med-063014-124936. PMID: 26768242

Diagnosis

Leisman S
Adv Chronic Kidney Dis 2020 Jan;27(1):50-55. doi: 10.1053/j.ackd.2019.08.002. PMID: 32147002
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
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
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
Beckett KR, Moriarity AK, Langer JM
Radiographics 2015 Oct;35(6):1738-50. doi: 10.1148/rg.2015150033. PMID: 26466182

Therapy

Starekova J, Pirasteh A, Reeder SB
AJR Am J Roentgenol 2024 Sep;223(3):e2330036. Epub 2023 Nov 15 doi: 10.2214/AJR.23.30036. PMID: 37850581
Leisman S
Adv Chronic Kidney Dis 2020 Jan;27(1):50-55. doi: 10.1053/j.ackd.2019.08.002. PMID: 32147002
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
Todd DJ, Kay J
Annu Rev Med 2016;67:273-91. doi: 10.1146/annurev-med-063014-124936. PMID: 26768242
Beckett KR, Moriarity AK, Langer JM
Radiographics 2015 Oct;35(6):1738-50. doi: 10.1148/rg.2015150033. PMID: 26466182

Prognosis

Mathur M, Jones JR, Weinreb JC
Radiographics 2020 Jan-Feb;40(1):153-162. Epub 2019 Dec 6 doi: 10.1148/rg.2020190110. PMID: 31809230
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
Galperin TA, Cronin AJ, Leslie KS
Clin J Am Soc Nephrol 2014 Jan;9(1):201-18. Epub 2013 Oct 10 doi: 10.2215/CJN.05900513. PMID: 24115194Free PMC Article
Igreja AC, Mesquita Kde C, Cowper SE, Costa IM
An Bras Dermatol 2012 Jul-Aug;87(4):597-607. doi: 10.1590/s0365-05962012000400013. PMID: 22892775

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
Blaha T, Nigwekar S, Combs S, Kaw U, Krishnappa V, Raina R
Hemodial Int 2019 Jan;23(1):3-18. Epub 2018 Dec 6 doi: 10.1111/hdi.12689. PMID: 30520561
Ramalho M, Ramalho J, Burke LM, Semelka RC
Adv Chronic Kidney Dis 2017 May;24(3):138-146. doi: 10.1053/j.ackd.2017.03.004. PMID: 28501075
Martin DR
Pediatr Radiol 2008 Jan;38 Suppl 1:S125-9. Epub 2007 Dec 11 doi: 10.1007/s00247-007-0589-8. PMID: 18071694
Grobner T, Prischl FC
Kidney Int 2007 Aug;72(3):260-4. Epub 2007 May 16 doi: 10.1038/sj.ki.5002338. PMID: 17507905

Recent systematic reviews

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
Mazhar SM, Shiehmorteza M, Kohl CA, Middleton MS, Sirlin CB
J Magn Reson Imaging 2009 Dec;30(6):1313-22. doi: 10.1002/jmri.21983. PMID: 19937937Free PMC Article
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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