Nephrogenic Systemic Fibrosis Differential Diagnoses

1. Tweedle MF. Science to Practice: Will Gadolinium Chelates Be Replaced by Iron Chelates in MR Imaging?. Radiology. 2018 Feb. 286 (2):409-411. [QxMD MEDLINE Link].

2. Wahba IM, Simpson EL, White K. Gadolinium is not the only trigger for nephrogenic systemic fibrosis: insights from two cases and review of the recent literature. Am J Transplant. 2007 Oct. 7(10):2425-32. [QxMD MEDLINE Link].

3. Lohani S, Golenbiewski J, Swami A, Halalau A. A unique case of nephrogenic systemic fibrosis from gadolinium exposure in a patient with normal eGFR. BMJ Case Rep. 2017 Oct 11. 2017:[QxMD MEDLINE Link].

4. Marckmann P, Skov L, Rossen K, et al. Nephrogenic systemic fibrosis: suspected causative role of gadodiamide used for contrast-enhanced magnetic resonance imaging. J Am Soc Nephrol. 2006 Sep. 17(9):2359-62. [QxMD MEDLINE Link].

5. Ross C, De Rosa N, Marshman G, Astill D. Nephrogenic systemic fibrosis in a gadolinium-naïve patient: successful treatment with oral sirolimus. Australas J Dermatol. 2015 Aug. 56 (3):e59-62. [QxMD MEDLINE Link].

6. Larson KN, Gagnon AL, Darling MD, Patterson JW, Cropley TG. Nephrogenic Systemic Fibrosis Manifesting a Decade After Exposure to Gadolinium. JAMA Dermatol. 2015 Oct. 151 (10):1117-20. [QxMD MEDLINE Link].

7. Bruce R, Wentland AL, Haemel AK, Garrett RW, Sadowski DR, Djamali A, et al. Incidence of Nephrogenic Systemic Fibrosis Using Gadobenate Dimeglumine in 1423 Patients With Renal Insufficiency Compared With Gadodiamide. Invest Radiol. 2016 Nov. 51 (11):701-705. [QxMD MEDLINE Link].

8. Soyer P, Dohan A, Patkar D, Gottschalk A. Observational study on the safety profile of gadoterate meglumine in 35,499 patients: The SECURE study. J Magn Reson Imaging. 2017 Apr. 45 (4):988-997. [QxMD MEDLINE Link].

9. Idée JM, Fretellier N, Robic C, Corot C. The role of gadolinium chelates in the mechanism of nephrogenic systemic fibrosis: A critical update. Crit Rev Toxicol. 2014 Nov. 44(10):895-913. [QxMD MEDLINE Link].

10. Cowper SE, Kuo PH, Bucala R. Nephrogenic systemic fibrosis and gadolinium exposure: association and lessons for idiopathic fibrosing disorders. Arthritis Rheum. 2007 Oct. 56(10):3173-5. [QxMD MEDLINE Link].

11. Yokoyama Y, Takeuchi Y, Monma F, Suzuki T, Ishikawa O Motegi S, Okada E, et al. Role of endothelin-1/endothelin receptor signaling in fibrosis and calcification in nephrogenic systemic fibrosis. Exp Dermatol. 2014 Sep. 23(9):664-9. [QxMD MEDLINE Link].

12. Wermuth PJ, Jimenez SA. Gadolinium Compounds Signaling through TLR 4 and TLR 7 in Normal Human Macrophages: Establishment of a Proinflammatory Phenotype and Implications for the Pathogenesis of Nephrogenic Systemic Fibrosis. J Immunol. 2012 Jul 1. 189:318-27. [QxMD MEDLINE Link].

13. Thomsen HS, Marckmann P, Logager VB. Nephrogenic systemic fibrosis (NSF): a late adverse reaction to some of the gadolinium based contrast agents. Cancer Imaging. 2007 Sep 24. 7:130-7. [QxMD MEDLINE Link].

14. Bongartz G, Kucharczyk W. Nephrogenic systemic fibrosis: Summary of the special symposium. J Magn Reson Imaging. 2007. 26:1179-81.

15. Thomsen HS, Morcos SK, Almén T, Bellin MF, Bertolotto M, Bongartz G, et al. Nephrogenic systemic fibrosis and gadolinium-based contrast media: updated ESUR Contrast Medium Safety Committee guidelines. Eur Radiol. 2013 Feb. 23:307-18. [QxMD MEDLINE Link].

16. Taupitz M, Stolzenburg N, Ebert M, Schnorr J, Hauptmann R, Kratz H, et al. Gadolinium-containing magnetic resonance contrast media: investigation on the possible transchelation of Gd(3+) to the glycosaminoglycan heparin. Contrast Media Mol Imaging. 2013. 8:108-16. [QxMD MEDLINE Link].

17. Goveia M, Chan BP, Patel PR. Evaluating the role of recombinant erythropoietin in nephrogenic systemic fibrosis. J Am Acad Dermatol. 2007 Oct. 57(4):725-7. [QxMD MEDLINE Link].

18. Saab G. Epoetin and nephrogenic systemic fibrosis. J Am Acad Dermatol. 2008 Jan. 58(1):174-5. [QxMD MEDLINE Link].

19. Gibson SE, Farver CF, Prayson RA. Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature. Arch Pathol Lab Med. 2006 Feb. 130(2):209-12. [QxMD MEDLINE Link].

20. Brown H. Gadodiamide, NSF: Direct Link Identified. Skin and Allergy News. 2007 Dec.

21. Parsons AC, Yosipovitch G, Sheehan DJ, Sangueza OP, Greenberg CS, Sane DC. Transglutaminases: the missing link in nephrogenic systemic fibrosis. Am J Dermatopathol. 2007 Oct. 29(5):433-6. [QxMD MEDLINE Link].

22. Edward M, Fitzgerald L, Thind C, Leman J, Burden AD. Cutaneous mucinosis associated with dermatomyositis and nephrogenic fibrosing dermopathy: fibroblast hyaluronan synthesis and the effect of patient serum. Br J Dermatol. 2007 Mar. 156(3):473-9. [QxMD MEDLINE Link].

23. Reimer P, Vosshenrich R. Contrast agents in radiology : Current agents approved, recommendations, and safety aspects. Radiologe. 2013 Jan 24. [QxMD MEDLINE Link].

24. Bose C, Megyesi JK, Shah SV, Hiatt KM, Hall KA, Karaduta O, et al. Evidence Suggesting a Role of Iron in a Mouse Model of Nephrogenic Systemic Fibrosis. PLoS One. 2015. 10 (8):e0136563. [QxMD MEDLINE Link].

25. Mackay-Wiggan JM, Cohen DJ, Hardy MA, Knobler EH, Grossman ME. Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease). J Am Acad Dermatol. 2003 Jan. 48(1):55-60. [QxMD MEDLINE Link].

26. McNeill AM, Barr RJ. Scleromyxedema-like fibromucinosis in a patient undergoing hemodialysis. Int J Dermatol. 2002 Jun. 41(6):364-7. [QxMD MEDLINE Link].

27. Jimenez SA, Artlett CM, Sandorfi N, et al. Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy): study of inflammatory cells and transforming growth factor beta1 expression in affected skin. Arthritis Rheum. 2004 Aug. 50(8):2660-6. [QxMD MEDLINE Link].

28. Ortonne N, Lipsker D, Chantrel F, Boehm N, Grosshans E, Cribier B. Presence of CD45RO+ CD34+ cells with collagen synthesis activity in nephrogenic fibrosing dermopathy: a new pathogenic hypothesis. Br J Dermatol. 2004 May. 150(5):1050-2. [QxMD MEDLINE Link].

29. Kucher C, Xu X, Pasha T, Elenitsas R. Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. J Cutan Pathol. 2005 Aug. 32(7):484-90. [QxMD MEDLINE Link].

30. Saab G, Cheng S. Nephrogenic systemic fibrosis: a nephrologist's perspective. Hemodial Int. 2007 Oct. 11 Suppl 3:S2-6. [QxMD MEDLINE Link].

31. Gadolinium-based Contrast Agents: Class Labeling Change - Risk of Nephrogenic Systemic Fibrosis. US Food and Drug Administration. September 9, 2010. Available at http://www.fda.gov/Safety/MedWatch/SafetyInformation/SafetyAlertsforHumanMedicalProducts/ucm225375.htm.

32. Deo A, Fogel M, Cowper SE. Nephrogenic systemic fibrosis: a population study examining the relationship of disease development to gadolinium exposure. Clin J Am Soc Nephrol. 2007 Mar. 2(2):264-7. [QxMD MEDLINE Link].

33. Todd DJ, Kagan A, Chibnik LB, Kay J. Cutaneous changes of nephrogenic systemic fibrosis: predictor of early mortality and association with gadolinium exposure. Arthritis Rheum. 2007 Oct. 56(10):3433-41. [QxMD MEDLINE Link].

34. Hubbard V, Davenport A, Jarmulowicz M, Rustin M. Scleromyxoedema-like changes in four renal dialysis patients. Br J Dermatol. 2003 Mar. 148(3):563-8. [QxMD MEDLINE Link].

35. Khor LK, Tan KB, Loi HY, Lu SJ. Nephrogenic Systemic Fibrosis in a Patient With Renal Failure Demonstrating a "Reverse Superscan" on Bone Scintigraphy. Clin Nucl Med. 2013 Jan 24.:[QxMD MEDLINE Link].

36. Elmholdt TR, Olesen AB, Jørgensen B, Kvist S, Skov L, Thomsen HS, et al. Nephrogenic systemic fibrosis in denmark- a nationwide investigation. PLoS One. 2013 Dec 9. 8(12):e82037. [QxMD MEDLINE Link].

37. Amet S, Launay-Vacher V, Clément O, Frances C, Tricotel A, Stengel B, et al. Incidence of nephrogenic systemic fibrosis in patients undergoing dialysis after contrast-enhanced magnetic resonance imaging with gadolinium-based contrast agents: the prospective fibrose nephrogenique systémique study. Invest Radiol. 2014 Feb. 49(2):109-15. [QxMD MEDLINE Link].

38. Cowper SE, Su LD, Bhawan J, Robin HS, LeBoit PE. Nephrogenic fibrosing dermopathy. Am J Dermatopathol. 2001 Oct. 23(5):383-93. [QxMD MEDLINE Link].

39. Jan F, Segal JM, Dyer J, LeBoit P, Siegfried E, Frieden IJ. Nephrogenic fibrosing dermopathy: two pediatric cases. J Pediatr. 2003 Nov. 143(5):678-81. [QxMD MEDLINE Link].

40. Weller A, Barber JL, Olsen OE. Gadolinium and nephrogenic systemic fibrosis: an update. Pediatr Nephrol. 2013 Oct 22.

41. Hancox JG, Mengesha YM, Sangueza OP, Yosipovitch G. Nephrogenic fibrosing dermopathy after five days of hemodialysis. J Drugs Dermatol. 2003 Oct. 2(5):550-3. [QxMD MEDLINE Link].

42. Ruiz-Genao DP, Pascual-Lopez MP, Fraga S, Aragues M, Garcia-Diez A. Osseous metaplasia in the setting of nephrogenic fibrosing dermopathy. J Cutan Pathol. 2005 Feb. 32(2):172-5. [QxMD MEDLINE Link].

43. Obermoser G, Emberger M, Wieser M, Zelger B. Nephrogenic fibrosing dermopathy in two patients with systemic lupus erythematosus. Lupus. 2004. 13(8):609-12. [QxMD MEDLINE Link].

44. Levine JM, Taylor RA, Elman LB, et al. Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy). Muscle Nerve. 2004 Nov. 30(5):569-77. [QxMD MEDLINE Link].

45. Hauser C, Kaya G, Chizzolini C. Nephrogenic fibrosing dermopathy in a renal transplant recipient with tubulointerstitial nephritis and uveitis. Dermatology. 2004. 209(1):50-2. [QxMD MEDLINE Link].

46. Glaich AS, Martinelli PT, Markus RF, Hsu S. Generalized elastolysis following nephrogenic fibrosing dermopathy. J Am Acad Dermatol. 2005 Jul. 53(1):174-6. [QxMD MEDLINE Link].

47. Kucher C, Steere J, Elenitsas R, Siegel DL, Xu X. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure. J Am Acad Dermatol. 2006 Feb. 54(2 Suppl):S31-4. [QxMD MEDLINE Link].

48. Edward M, Fitzgerald L, Thind C, Leman J, Burden AD. Cutaneous mucinosis associated with dermatomyositis and nephrogenic fibrosing dermopathy: fibroblast hyaluronan synthesis and the effect of patient serum. Br J Dermatol. 2007 Mar. 156(3):473-9. [QxMD MEDLINE Link].

49. Saenz A, Mandal R, Kradin R, Hedley-Whyte ET. Nephrogenic fibrosing dermopathy with involvement of the dura mater. Virchows Arch. 2006 Sep. 449(3):389-91. [QxMD MEDLINE Link].

50. Naylor E, Hu S, Robinson-Bostom L. Nephrogenic systemic fibrosis with septal panniculitis mimicking erythema nodosum. J Am Acad Dermatol. 2008 Jan. 58(1):149-50. [QxMD MEDLINE Link].

51. Keyrouz S, Rudnicki SA. Neuromuscular involvement in nephrogenic systemic fibrosis. J Clin Neuromuscul Dis. 2007 Dec. 9(2):297-302. [QxMD MEDLINE Link].

52. Sanchez-Ross M, Snyder R, Colome-Grimmer MI, Blumberg M, Huttenbach Y, Raimer S. Nephrogenic fibrosing dermopathy in a patient with systemic lupus erythematosus and acute lupus nephritis. Pediatr Dermatol. 2007 Sep-Oct. 24(5):E36-9. [QxMD MEDLINE Link].

53. Goddard DS, Magee CC, Lazar AJ, Miller DM. Nephrogenic fibrosing dermopathy with recurrence after allograft failure. J Am Acad Dermatol. 2007 May. 56(5 Suppl):S109-11. [QxMD MEDLINE Link].

54. High WA, Ayers RA, Cowper SE. Gadolinium is quantifiable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol. 2007 Apr. 56(4):710-2. [QxMD MEDLINE Link].

55. Gathings RM, Reddy R, Santa Cruz D, Brodell RT. Gadolinium-Associated Plaques: A New, Distinctive Clinical Entity. JAMA Dermatol. 2014 Nov 12. [QxMD MEDLINE Link].

56. Ross C, De Rosa N, Marshman G, Astill D. Nephrogenic systemic fibrosis in a gadolinium-naïve patient: Successful treatment with oral sirolimus. Australas J Dermatol. 2014 Oct 21. [QxMD MEDLINE Link].

57. Smorodinsky E, Ansdell DS, Foster ZW, Mazhar SM, Cruite I, Wolfson T, et al. Risk of nephrogenic systemic fibrosis is low in patients with chronic liver disease exposed to gadolinium-based contrast agents. J Magn Reson Imaging. 2014 May 9. [QxMD MEDLINE Link].

58. Nguyen CV, Boyle AM, Lee RA. Perforating osteoma cutis in the setting of longstanding nephrogenic systemic fibrosis. Int J Dermatol. 2014 Jun 25. [QxMD MEDLINE Link].

59. Solomon GJ, Wu E, Rosen PP. Nephrogenic systemic fibrosis mimicking inflammatory breast carcinoma. Arch Pathol Lab Med. 2007 Jan. 131(1):145-8. [QxMD MEDLINE Link].

60. Thakral C, Abraham JL. Automated scanning electron microscopy and x-ray microanalysis for in situ quantification of gadolinium deposits in skin. J Electron Microsc (Tokyo). 2007 Oct. 56(5):181-7. [QxMD MEDLINE Link].

61. Christensen KN, Lee CU, Hanley MM, Leung N, Moyer TP, Pittelkow MR. Quantification of gadolinium in fresh skin and serum samples from patients with nephrogenic systemic fibrosis. J Am Acad Dermatol. 2011 Jan. 64(1):91-6. [QxMD MEDLINE Link].

62. Kintossou R, D'Incan M, Chauveau D, et al. [Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis: role of gadolinium?]. Ann Dermatol Venereol. 2007 Aug-Sep. 134(8-9):667-71. [QxMD MEDLINE Link].

63. Elmholdt TR, Buus NH, Ramsing M, Olesen AB. Antifibrotic effect after low-dose imatinib mesylate treatment in patients with nephrogenic systemic fibrosis: an open-label non-randomized, uncontrolled clinical trial. J Eur Acad Dermatol Venereol. 2011 Dec 20. [QxMD MEDLINE Link].

64. Schmook T, Budde K, Ulrich C, Neumayer HH, Fritsche L, Stockfleth E. Successful treatment of nephrogenic fibrosing dermopathy in a kidney transplant recipient with photodynamic therapy. Nephrol Dial Transplant. 2005 Jan. 20(1):220-2. [QxMD MEDLINE Link].

65. Kafi R, Fisher GJ, Quan T, et al. UV-A1 phototherapy improves nephrogenic fibrosing dermopathy. Arch Dermatol. 2004 Nov. 140(11):1322-4. [QxMD MEDLINE Link].

66. Lauchli S, Zortea-Caflisch C, Nestle FO, Burg G, Kempf W. Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis. Dermatology. 2004. 208(3):278-80. [QxMD MEDLINE Link].

67. Chung HJ, Chung KY. Nephrogenic fibrosing dermopathy: response to high-dose intravenous immunoglobulin. Br J Dermatol. 2004 Mar. 150(3):596-7. [QxMD MEDLINE Link].

68. Wahba IM, White K, Meyer M, Simpson EL. The case for ultraviolet light therapy in nephrogenic fibrosing dermopathy--report of two cases and review of the literature. Nephrol Dial Transplant. 2007 Feb. 22(2):631-6. [QxMD MEDLINE Link].

69. Richmond H, Zwerner J, Kim Y, Fiorentino D. Nephrogenic systemic fibrosis: relationship to gadolinium and response to photopheresis. Arch Dermatol. 2007 Aug. 143(8):1025-30. [QxMD MEDLINE Link].

70. Yerram P, Saab G, Karuparthi PR, Hayden MR, Khanna R. Nephrogenic systemic fibrosis: a mysterious disease in patients with renal failure--role of gadolinium-based contrast media in causation and the beneficial effect of intravenous sodium thiosulfate. Clin J Am Soc Nephrol. 2007 Mar. 2(2):258-63. [QxMD MEDLINE Link].

71. Kreuter A, Gambichler T, Weiner SM, Schieren G. Limited effects of UV-A1 phototherapy in 3 patients with nephrogenic systemic fibrosis. Arch Dermatol. 2008 Nov. 144(11):1527-9. [QxMD MEDLINE Link].

72. Zou Z, Zhang HL, Roditi GH, Leiner T, Kucharczyk W, Prince MR. Nephrogenic systemic fibrosis: review of 370 biopsy-confirmed cases. JACC Cardiovasc Imaging. 2011 Nov. 4:1206-16. [QxMD MEDLINE Link].

73. Lancelot E, Raynaud JS, Desché P. Current and Future MR Contrast Agents: Seeking a Better Chemical Stability and Relaxivity for Optimal Safety and Efficacy. Invest Radiol. 2020 Sep. 55 (9):578-588. [QxMD MEDLINE Link].

74. Xue X, Bo R, Qu H, Jia B, Xiao W, Yuan Y, et al. A nephrotoxicity-free, iron-based contrast agent for magnetic resonance imaging of tumors. Biomaterials. 2020 Oct. 257:120234. [QxMD MEDLINE Link].

75. Lunyera J, Mohottige D, Alexopoulos AS, Campbell H, Cameron CB, Sagalla N, et al. Risk for Nephrogenic Systemic Fibrosis After Exposure to Newer Gadolinium Agents: A Systematic Review. Ann Intern Med. 2020 Jul 21. 173 (2):110-119. [QxMD MEDLINE Link].

76. Lersy F, Boulouis G, Clément O, Desal H, Anxionnat R, Berge J, et al. Consensus Guidelines of the French Society of Neuroradiology (SFNR) on the use of Gadolinium-Based Contrast agents (GBCAs) and related MRI protocols in Neuroradiology. J Neuroradiol. 2020 Jun 18. [QxMD MEDLINE Link].

77. Marckmann P, Nielsen AH, Sloth JJ. Possibly enhanced Gd excretion in dialysate, but no major clinical benefit of 3-5 months of treatment with sodium thiosulfate in late stages of nephrogenic systemic fibrosis. Nephrol Dial Transplant. 2008 Oct. 23(10):3280-2. [QxMD MEDLINE Link].

Shawn Cowper, MD Director, NSF Registry Project, Associate Professor, Departments of Dermatology and Pathology, Yale University School of Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Bracco pharmaceuticals; Ecron-Acunova.

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Noah S Scheinfeld, JD, MD, FAAD † Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

• Print this section
• Print the entire contents of
• Print the entire contents of article