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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

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
Gadolinium-Induced Fibrosis.
Todd DJ, Kay J
Annu Rev Med 2016;67:273-91. doi: 10.1146/annurev-med-063014-124936. PMID: 26768242
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
Nephrogenic systemic fibrosis.
Weinreb JC, Kuo PH
Magn Reson Imaging Clin N Am 2009 Feb;17(1):159-67. doi: 10.1016/j.mric.2009.01.003. PMID: 19364606

Diagnosis

Gadolinium: pharmacokinetics and toxicity in humans and laboratory animals following contrast agent administration.
Davies J, Siebenhandl-Wolff P, Tranquart F, Jones P, Evans P
Arch Toxicol 2022 Feb;96(2):403-429. Epub 2022 Jan 8 doi: 10.1007/s00204-021-03189-8. PMID: 34997254Free PMC Article
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
Safe Use of Contrast Media: What the Radiologist Needs to Know.
Beckett KR, Moriarity AK, Langer JM
Radiographics 2015 Oct;35(6):1738-50. doi: 10.1148/rg.2015150033. PMID: 26466182
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

Therapy

Gadolinium: pharmacokinetics and toxicity in humans and laboratory animals following contrast agent administration.
Davies J, Siebenhandl-Wolff P, Tranquart F, Jones P, Evans P
Arch Toxicol 2022 Feb;96(2):403-429. Epub 2022 Jan 8 doi: 10.1007/s00204-021-03189-8. PMID: 34997254Free PMC Article
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
Gadolinium-Induced Fibrosis.
Todd DJ, Kay J
Annu Rev Med 2016;67:273-91. doi: 10.1146/annurev-med-063014-124936. PMID: 26768242
Safe Use of Contrast Media: What the Radiologist Needs to Know.
Beckett KR, Moriarity AK, Langer JM
Radiographics 2015 Oct;35(6):1738-50. doi: 10.1148/rg.2015150033. PMID: 26466182
Nephrogenic systemic fibrosis.
Weinreb JC, Kuo PH
Magn Reson Imaging Clin N Am 2009 Feb;17(1):159-67. doi: 10.1016/j.mric.2009.01.003. PMID: 19364606

Prognosis

Gadolinium Deposition and Nephrogenic Systemic Fibrosis: A Radiologist's Primer.
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
Skin manifestations of chronic kidney disease.
Robles-Mendez JC, Vazquez-Martinez O, Ocampo-Candiani J
Actas Dermosifiliogr 2015 Oct;106(8):609-22. Epub 2015 Jun 17 doi: 10.1016/j.ad.2015.05.007. PMID: 26093993
Nephrogenic fibrosing dermopathy.
Ragunatha S, Palit A, Inamadar AC, Madraki RM, Yelikar BR
Indian J Dermatol Venereol Leprol 2009 Jan-Feb;75(1):63-7. doi: 10.4103/0378-6323.45224. PMID: 19172035
Scleroderma-like fibrosing disorders.
Boin F, Hummers LK
Rheum Dis Clin North Am 2008 Feb;34(1):199-220; ix. doi: 10.1016/j.rdc.2007.11.001. PMID: 18329541Free PMC Article
Nephrogenic fibrosing dermopathy: the first 6 years.
Cowper SE
Curr Opin Rheumatol 2003 Nov;15(6):785-90. doi: 10.1097/00002281-200311000-00017. PMID: 14569211

Clinical prediction guides

Gadolinium contrast agents - are they really safe?
Malikova H, Holesta M
J Vasc Access 2017 Mar 28;18(Suppl. 2):1-7. Epub 2017 Mar 21 doi: 10.5301/jva.5000713. PMID: 28362042
Gadolinium toxicity and treatment.
Ramalho J, Ramalho M, Jay M, Burke LM, Semelka RC
Magn Reson Imaging 2016 Dec;34(10):1394-1398. Epub 2016 Sep 28 doi: 10.1016/j.mri.2016.09.005. PMID: 27693607
Nephrogenic systemic fibrosis: an emerging threat among renal patients.
DeHoratius DM, Cowper SE
Semin Dial 2006 May-Jun;19(3):191-4. doi: 10.1111/j.1525-139X.2006.00152.x. PMID: 16689966
Nephrogenic fibrosing dermopathy.
Gambichler T, Paech V, Kreuter A, Wilmert M, Altmeyer P, Stücker M
Clin Exp Dermatol 2004 May;29(3):258-60. doi: 10.1111/j.0307-6938.2004.01474.x. PMID: 15115505
Nephrogenic fibrosing dermopathy: the first 6 years.
Cowper SE
Curr Opin Rheumatol 2003 Nov;15(6):785-90. doi: 10.1097/00002281-200311000-00017. PMID: 14569211

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 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
A Systematic Review of 639 Patients with Biopsy-confirmed Nephrogenic Systemic Fibrosis.
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
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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