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Nephrogenic fibrosing dermopathy

MedGen UID:
301436
•Concept ID:
C1619692
•
Disease or Syndrome
Synonyms: Dermopathies, Nephrogenic Fibrosing; Dermopathy, Nephrogenic Fibrosing; Fibroses, Nephrogenic Systemic; Fibrosing Dermopathies, Nephrogenic; Fibrosing Dermopathy, Nephrogenic; Fibrosis, Nephrogenic Systemic; Nephrogenic Fibrosing Dermopathies; Nephrogenic Fibrosing Dermopathy; Nephrogenic Systemic Fibroses; Nephrogenic Systemic Fibrosis; Systemic Fibroses, Nephrogenic; Systemic Fibrosis, Nephrogenic
SNOMED CT: Nephrogenic fibrosing dermopathy (424114000); NSF - Nephrogenic fibrosing dermopathy (424114000)

Definition

A rare disorder characterized by fibrosis of the skin and internal organs. It occurs in patients with renal failure who had imaging studies performed using gadolinium. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNephrogenic fibrosing dermopathy
Follow this link to review classifications for Nephrogenic fibrosing dermopathy in Orphanet.

Professional guidelines

PubMed

European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton CP, Rudnicka L, Frasin LA, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt JM, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte NJ, Krieg T
J Eur Acad Dermatol Venereol 2017 Oct;31(10):1581-1594. Epub 2017 Aug 8 doi: 10.1111/jdv.14466. PMID: 28786499

Recent clinical studies

Etiology

Update on Gadolinium-Based Contrast Agent Safety, From the AJR Special Series on Contrast Media.
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
Gadolinium-based contrast agents in children.
Rozenfeld MN, Podberesky DJ
Pediatr Radiol 2018 Aug;48(9):1188-1196. Epub 2018 Aug 4 doi: 10.1007/s00247-018-4165-1. PMID: 30078039
Pathophysiology of gadolinium-associated systemic fibrosis.
Wagner B, Drel V, Gorin Y
Am J Physiol Renal Physiol 2016 Jul 1;311(1):F1-F11. Epub 2016 May 4 doi: 10.1152/ajprenal.00166.2016. PMID: 27147669Free PMC Article
Gadolinium-Induced Fibrosis.
Todd DJ, Kay J
Annu Rev Med 2016;67:273-91. doi: 10.1146/annurev-med-063014-124936. PMID: 26768242
Scleroderma mimics.
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103

Diagnosis

Imaging patients with renal impairment.
Mathur M, Weinreb JC
Abdom Radiol (NY) 2016 Jun;41(6):1108-21. doi: 10.1007/s00261-016-0709-8. PMID: 27015867
Nephrogenic systemic fibrosis.
Kaewlai R, Abujudeh H
AJR Am J Roentgenol 2012 Jul;199(1):W17-23. doi: 10.2214/AJR.11.8144. PMID: 22733927
Scleroderma mimics.
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103
Low dose CE-MRA.
Nael K, Moriarty JM, Finn JP
Eur J Radiol 2011 Oct;80(1):2-8. Epub 2011 Apr 1 doi: 10.1016/j.ejrad.2011.01.092. PMID: 21458187
Nephrogenic systemic fibrosis.
Vosshenrich R, Reimer P
Vasa 2009 Feb;38(1):31-8. doi: 10.1024/0301-1526.38.1.31. PMID: 19229801

Therapy

Update on Gadolinium-Based Contrast Agent Safety, From the AJR Special Series on Contrast Media.
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
Gadolinium-based contrast agents in children.
Rozenfeld MN, Podberesky DJ
Pediatr Radiol 2018 Aug;48(9):1188-1196. Epub 2018 Aug 4 doi: 10.1007/s00247-018-4165-1. PMID: 30078039
Gadolinium-Induced Fibrosis.
Todd DJ, Kay J
Annu Rev Med 2016;67:273-91. doi: 10.1146/annurev-med-063014-124936. PMID: 26768242
Low dose CE-MRA.
Nael K, Moriarty JM, Finn JP
Eur J Radiol 2011 Oct;80(1):2-8. Epub 2011 Apr 1 doi: 10.1016/j.ejrad.2011.01.092. PMID: 21458187
Nephrogenic systemic fibrosis.
Vosshenrich R, Reimer P
Vasa 2009 Feb;38(1):31-8. doi: 10.1024/0301-1526.38.1.31. PMID: 19229801

Prognosis

Appropriate and safe use of diagnostic imaging.
Crownover BK, Bepko JL
Am Fam Physician 2013 Apr 1;87(7):494-501. PMID: 23547591
Nephrogenic systemic fibrosis and gadolinium-based contrast media: updated ESUR Contrast Medium Safety Committee guidelines.
Thomsen HS, Morcos SK, Almén T, Bellin MF, Bertolotto M, Bongartz G, Clement O, Leander P, Heinz-Peer G, Reimer P, Stacul F, van der Molen A, Webb JA; ESUR Contrast Medium Safety Committee
Eur Radiol 2013 Feb;23(2):307-18. Epub 2012 Aug 4 doi: 10.1007/s00330-012-2597-9. PMID: 22865271
Nephrogenic systemic fibrosis: concepts and perspectives.
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
Non-contrast-enhanced magnetic resonance angiography: techniques and applications.
Blankholm AD, Ringgaard S
Expert Rev Cardiovasc Ther 2012 Jan;10(1):75-88. doi: 10.1586/erc.11.176. PMID: 22149527
Nephrogenic systemic fibrosis in liver disease: a systematic review.
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

Clinical prediction guides

Risks and Options With Gadolinium-Based Contrast Agents in Patients With CKD: A Review.
Rudnick MR, Wahba IM, Leonberg-Yoo AK, Miskulin D, Litt HI
Am J Kidney Dis 2021 Apr;77(4):517-528. Epub 2020 Aug 27 doi: 10.1053/j.ajkd.2020.07.012. PMID: 32861792
Renal function, nephrogenic systemic fibrosis and other adverse reactions associated with gadolinium-based contrast media.
Canga A, Kislikova M, Martínez-Gálvez M, Arias M, Fraga-Rivas P, Poyatos C, de Francisco AL
Nefrologia 2014;34(4):428-38. doi: 10.3265/Nefrologia.pre2014.Apr.12375. PMID: 25036056
Appropriate and safe use of diagnostic imaging.
Crownover BK, Bepko JL
Am Fam Physician 2013 Apr 1;87(7):494-501. PMID: 23547591
Non-contrast-enhanced magnetic resonance angiography: techniques and applications.
Blankholm AD, Ringgaard S
Expert Rev Cardiovasc Ther 2012 Jan;10(1):75-88. doi: 10.1586/erc.11.176. PMID: 22149527
Nephrogenic systemic fibrosis: a gadolinium-associated fibrosing disorder in patients with renal dysfunction.
Kay J
Ann Rheum Dis 2008 Dec;67 Suppl 3:iii66-9. doi: 10.1136/ard.2008.102475. PMID: 19022818

Recent systematic reviews

Adverse Events Associated with Intra-Arterial Administration of Gadolinium-Based Contrast Agents: A Systematic Review and Meta-Analysis.
MacLeod CA, Gauthier I, Davenport MS, McGrath TA, Khan F, Dos Santos MP, McInnes MDF, Schieda N
J Vasc Interv Radiol 2023 Apr;34(4):568-577.e10. Epub 2022 Dec 2 doi: 10.1016/j.jvir.2022.11.022. PMID: 36464013
Risk of Nephrogenic Systemic Fibrosis in Stage 4 and 5 Chronic Kidney Disease Following Group II Gadolinium-based Contrast Agent Administration: Subanalysis by Chronic Kidney Disease Stage.
Shankar PR, Davenport MS
Radiology 2020 Nov;297(2):447-448. Epub 2020 Aug 18 doi: 10.1148/radiol.2020201492. PMID: 32808890
Risk of Nephrogenic Systemic Fibrosis in Patients With Stage 4 or 5 Chronic Kidney Disease Receiving a Group II Gadolinium-Based Contrast Agent: A Systematic Review and Meta-analysis.
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
Nephrogenic systemic fibrosis in liver disease: a systematic review.
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
Gadolinium-based contrast agents and nephrogenic systemic fibrosis: a systematic review and meta-analysis.
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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