The International Center for Nephrogenic Systemic Fibrosis Research (ICNSFR)

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By Shawn E. Cowper, MD
Associate Professor of Dermatology and Pathology
Yale University

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1. Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE. Scleromyxoedema-like cutaneous disease in renal-dialysis patients. The Lancet 356(9234), 16 September 2000, 1000-1
2. Cowper SE, Su L, Robin H, Bhawan J, LeBoit PE. Nephrogenic Fibrosing Dermopathy. Amer J Dermatopathol 23(5): 383-393, 2001
3. Fibrosing skin condition among patients with renal disease—United States and Europe 1997-2002. MMWR/Morb Mortal Wkly Rep 51:25-6, 2002
4. McNeill AM, Barr RJ. Scleromyxedema-like fibromucinosis in a patient undergoing hemodialysis. International Jour Dermatol 41(6): 364-7, 2002
5. Bates B. New Dermopathy Seen in Kidney Disease Patients, eSkin and Allergy News 33(9) September, 2002
6. Bardin T. Musculoskeletal manifestations of chronic renal failure, Curr Opin Rheumatol.15(1): 48-54.Jan 2003 (Mentions NFD briefly, no discussion)
7. Mackay-Wiggan JM, Cohen DJ, Hardy MA, Knobler EH, Grossman ME. Nephrogenic fibrosing dermopathy (scleromyxedema-like illness of renal disease, Jour Amer Acad Dermatol.48(1): 55-60.Jan 2003
8. Streams BN, Liu V, Liegeois N, Moschella SM. Clinical and pathological features of nephrogenic fibrosing dermopathy, Jour Amer Acad Dermatol.48(1): 42-7.Jan 2003
9. Hubbard V, Davenport A, Jarmulowicz M, Rustin M. Scleromyxoedema-like change in four renal dialysis patients, Br J Dermatol. 148(3): 563-8.Mar 2003
10. Swartz RD, Crofford LJ, Phan SH, Ike RW, Su LD. Nephrogenic Fibrosing Dermopathy: A novel cutaneous fibrosing disorder in patients with renal failure, Am J Med. 114(7): 563-72.May 2003
11. Perazella MA, Ishibe S, Perazella MA, Reilly RF. Nephrogenic Fibrosing Dermopathy: an unusual skin condition associated with kidney disease, Semin Dial. 16(3): 276-80.May-June 2003
12. Baron PW, Cantos K, Hillebrand DJ, H KQ, Ojogho ON, Nehlsen-Cannarella S, Concepcion W. Nephrogenic Fibrosing Dermopathy After Liver Transplantation Successfully Treated With Plasmapheresis. Amer J Dermatopathol 25(3):204-209. June 2003
13. Ting WW, Stone MS, Madison KC, Kurtz K. Nephrogenic Fibrosing Dermopathy with Systemic Involvement. Archiv Dermatol 139(7):903-6. July 2003
14. Leboit PE. What nephrogenic fibrosing dermopathy might be. Archiv Dermatol 139(7):928-30. July 2003
15. Cowper SE, Bucala R. Nephrogenic Fibrosing Dermopathy: Suspect Identified, Motive Unclear. Amer J Dermatopathol 25(4):358. August 2003
16. Hancox JG, Mengesha YM, Sangueza OP, Yosipovitch G. Nephrogenic fibrosing dermopathy after five days of hemodialysis. J Drugs Dermatol. 2(5):550-3. October 2003.
17. Cowper SE. Nephrogenic Fibrosing Dermopathy: The First Six Years. Curr Opin Rheumatol.15(6):785-90. November 2003
18. Jan F, Segal JM, Dyer J, Leboit P, Siegfried E, Frieden IJ. Nephrogenic Fibrosing Dermopathy: Two Pediatric Cases. J Pediatr.143:678-81. November 2003
19. Engelen JW, Kooistra MP, Canninga-van Dijk MR, Toonstra J, Sigurdsson V. Nephrogenic Fibrosing Dermopathy [Dutch] Ned Tijdschr Geneeskd. 147(49):2435-8. Dec 2003
20. Dawn G, Holmes SC. Scleromyxoedema-like eruption following haemodialysis or Nephrogenic Fibrosing Dermopathy? [Letter] Br J Dermatol. 150(1):167-8. Jan 2004
21. Evenepoel P, Zeegers M, Segaert S, Claes K, Kuypers D, Maes B, Flamen P, Fransis S, Vanrenterghem Y. Nephrogenic Fibrosing Dermopathy: A Novel Disabling Disorder in Patients with Renal Failure. Nephrol Dial Transplant.19(2):469-73. February 2004
22. Jain SM, Wesson S, Hassanein A, Canova E, Hoy M, Fennell RS, Dharnidharka VR. Nephrogenic Fibrosing Dermopathy in pediatric patients. Pediatr Nephrol. February 2004
23. Edsall LC, English JC, Patterson JW. Calciphylaxis and metastatic calcification associated with nephrogenic fibrosing dermopathy. J Cutan Pathol. 31(3):247-53. March 2004
24. Hershko K, Hull C, Ettefagh L, Nedorost S, Dyson S, Horn T, Gilliam AC. A variant of nephrogenic fibrosing dermopathy with osteoclast-like giant cells: a syndrome of dysregulated matrix remodelling? J Cutan Pathol. 31(3):262-5. March 2004
25. Chiu H, Wells G, Carag H, Canova E, Firpi RJ. Nephrogenic fibrosing dermopathy: A rare entity in patients awaiting liver transplantation Liver Transpl. 10(3):465-6. March 2004
26. Chung HJ, Chung KY. Nephrogenic fibrosing dermopathy: response to high-dose intravenous immunoglobulin [Letter] Br J Dermatol. 150(3):596-7. March 2004
27. Quan TE, Cowper S, Wu SP, Bockenstedt LK, Bucala R. Circulating fibrocytes: Collagen-secreting cells of the peripheral blood. Int J Biochem Cell Biol. 36(4):598-606. April 2004
28. Gambichler T, Paech V, Kreuter A, Wilmert M, Altmeyer P, Stucker M. Nephrogenic fibrosing dermopathy Clin Exp Dermatol. 29(3):258-60. May 2004
29. Lauchli S, Zortea-Caflisch C, Nestle FO, Burg G, Kempf W. Nephrogenic fibrosing dermopathy treated with extracorporeal photopheresis Dermatology 208(3):278-80. May 2004
30. 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. 150(3):1050-2. May 2004
Hauser C, Kaya G, Chizzolini C. Nephrogenic fibrosing dermopathy in a renal transplant recipient with tubulointerstitial nephritis and uveitis. Dermatol. 209(1):50-2. July 2004
32. Tan AW, Tan SH, Lian TY, Ng SK. A case of nephrogenic fibrosing dermopathy. Ann Acad Med Singapore. 33(4):527-9. July 2004
*33. [SEE NOTE BELOW] Jimenez SA, Artlett CM, Sandorfi N, Derk C, Latinis K, Sawaya H, Haddad R, Shanahan JC. Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy): study of inflammatory cells and transforming growth factor beta1 expression in affected skin. Arthritis Rheum. 50(8):2660-6. August 2004
34. Taylor EN, Henderson JM, Rennke HG, Magee CC. Traumatic calcinosis cutis in a dialysis patient. Am J Kidney Dis. 44(2):e6-9. August 2004
35. Obermoser G, Emberger M, Wieser M, Zelger B. Nephrogenic fibrosing dermopathy in two patients with systemic lupus erythematosus. Lupus. 13(8):609-12. August 2004

*36. [SEE NOTE BELOW] Levine JM, Taylor RA, Elman LB, Bird SJ, Lavi E, Stolzenberg ED, McGarvey ML, Asbury AK, Jimenez SA. Involvement of skeletal muscle in dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy). Muscle Nerve. 30(5):569-77. November 2004
37. Gremmels JM, Kirk GA. Two Patients With Abnormal Skeletal Muscle Uptake of Tc-99m Hydroxymethylene Diphosphonate Following Liver Transplant: Nephrogenic Fibrosing Dermopathy and Graft vs Host Disease. Clin Nucl Med. 29(11):694-7. November 2004
38. Kafi R, Fisher GJ, Quan T, Shao Y, Wang R, Voorhees JJ, Kang S. UV-A1 phototherapy improves nephrogenic fibrosing dermopathy. Arch Derm. 140(11):1322-4. November 2004
39. Fazeli A, Lio PA, Liu V. Nephrogenic fibrosing dermopathy: are ACE inhibitors the missing link? [Letter] Arch Derm. 140(11):1401. November 2004
40. Moschella SL, Kay J, Mackool BT, Liu V. Case 35-2004: A 68-year-old man with end-stage renal disease and thickening of the skin. N Engl J Med. 351:2219-27. November 2004
41. Markus JS, James AJ, Nunez-Gussman JK, Sheehan AM, Fegan L, Hsu S. Nephrogenic fibrosing dermopathy. J Am Acad Dermatol. 52(1):166-7. January 2005
42. 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. 20(1):220-2. January 2005
43. 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. 32(2):172-5. February 2005

44. Gilliet M, Cozzio A, Burg G, Nestle FO. Successful treatment of three cases of nephrogenic fibrosing dermopathy with extracorporeal photopheresis. Br J Dermatol. 152(3):531-6. March 2005
45. Cowper SE, Bucala R, LeBoit PE. Comment on: Case 35:2004 Nephrogenic fibrosing dermopathy. [Letter] N Engl J Med. 352(16):1723-4. August 2005
46. Taylor RA, Levine JM, Jimenez SA. Comment on: Case 35:2004 Nephrogenic fibrosing dermopathy. [Letter] N Engl J Med. 352(16):1723-4. August 2005
47. Moschella SL, Kay J, Liu V. The authors reply: Case 35:2004 Nephrogenic fibrosing dermopathy.
[Letter] N Engl J Med. 352(16):1723-4. August 2005
48. Glaich AS, Martinelli PT, Markus RF, Hsu S. Generalized elastolysis following nephrogenic fibrosing dermopathy. J Am Acad Dermatol. 53(1):174-6. July 2005
49. Kucher C, Xu X, Pasha T, Elenitsas R. Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. J Cutan Pathol. 32(7):484-90. August 2005
50. Neudecker BA, Stern R, Mark LA, Steinberg S. Scleromyxedema-like lesions of patients in renal failure contain hyaluronan: a possible pathophysiological mechanism. J Cutan Pathol. 32(9):612-5. October 2005
51. Daram SR, Cortese CM, Bastani B. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis: report of a new case with literature review. Am J Kidney Dis. 46(4):754-9. October 2005
52. Cowper SE. Nephrogenic systemic fibrosis: the nosological and conceptual evolution of nephrogenic fibrosing dermopathy. Am J Kidney Dis. 46(4):763-5. October 2005
53. Dupont A, Majithia V, Ahmad S, McMurray R. Nephrogenic fibrosing dermopathy, a new mimicker of systemic sclerosis. Am J Med Sci. 330(4):192-4. October 2005
54. Dundova I, Treska V, Simanek V, Michal M. Nephrogenic fibrosing dermopathy: a case study. Transplant Proc. 37(10):4187-90. December 2005
55. Panda S, Bandyopadhyay D, Tarafder A. Nephrogenic fibrosing dermopathy: a series in a non-Western population. J Am Acad Dermatol. 54(1):155-9. January 2006
56. Cassis TB, Jackson JM, Sonnier GB, Callen JP. Nephrogenic fibrosing dermopathy in a patient with acute renal failure never requiring dialysis. Int J Dermatol. 45(1):56-9. January 2006
57. Grobner T. Gadolinium - a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? Nephrol Dial Transplant. 21(4):1104-8. Online publication January 2006
57e. Grobner T. Gadolinium—a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? [erratum] Nephrol. Dial. Transplant. 21:1745. June 2006
58. Shelekhova KV, Kazakov DV, Michal M. Nephrogenic fibrosing dermopathy [article in Russian]. Arkh Patol. 68(1):42-3. January/February 2006
59. 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. 54(2 Suppl):S31-4. February 2006
60. Gibson SE, Farver CF, Prayson RA. Multiorgan involvement in nephrogenic fibrosing dermopathy: an autopsy case and review of the literature. Arch Pathol Lab Med. 130(2):209-12. February 2006
61. Mendoza FA, Artlett CM, Sandorfi N, Latinis K, Piera-Velazquez S, Jimenez SA. Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature. Semin Arthritis Rheum. 35(4):238-49. February 2006
62. Cowper SE, Bucala R, Leboit PE. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis-setting the record straight. Semin Arthritis Rheum. 35(4):208-10. February 2006
63. George DE, Lu R, George SJ, Hsu S. Nephrogenic fibrosing dermopathy: case report and review. Dermatol Online J 12(3):7. March 2006
64. Cowper SE, Boyer PJ. Nephrogenic systemic fibrosis: an update. Curr Rheum Reports 8(2):151-7. April 2006
65. DiCarlo JB, Gupta EA, Solomon AR. A pediatric case of nephrogenic fibrosing dermopathy: improvement after combination therapy. J Am Acad Dermatol 54(5):914-6. May 2006
66. Dehoratius DM, Cowper SE. Nephrogenic systemic fibrosis: an emerging threat among renal patients. Sem in Dialysis 19(3):191-4. May/June 2006
67. Kim RH, Ma L, Hayat SQ, Ahmed MM. Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis in 2 patients with end-stage renal disease on hemodialysis. J Clin Rheumatol 12(3):134-6. June 2006
68. Swaminathan S, Leung N. Nephrogenic fibrosing dermopath
y: lessons from the past. Int J Dermatol 45(6):639-41. June 2006
69. Romanelli P, Bouzari N. New clinical syndromes in dermatology. Semin Cutan Med Surg. 25(2):79-86. June 2006
70. Maloo M, Abt P, Kashyap R, et al. Nephrogenic systemic fibrosis among liver transplant recipients: a single institution experience and topic update. Am J Transplant. 6(9):2212-7. September 2006. Epub June 2006
71. Auron A, Shao L, Warady BA. Nephrogenic fibrosing dermopathy in children. Pediatr Nephrol 21(9):1307-11. September 2006. Epub July 2006
72. Swaminathan S, Ahmed I, McCarthy JT, et al. Nephrogenic fibrosing dermopathy and high-dose erythropoietin therapy. Ann Intern Med 1;145(3):234-5. August 2006
73. Saenz A, Mandal R, Kradin R, Hedley-Whyte ET. Nephrogenic fibrosing dermopathy with involvement of the dura mater. Virchows Arch. 449(3):389-91. September 2006. Epub August 2006
74. 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. 17(9):2359-62. September 2006. Epub August 2006
75. Scheinfeld N. Nephrogenic fibrosing dermopathy: a comprehensive review for the dermatologist. Am J Clin Dermatol 7(4):237-4. August 2006
76. Lewis KG, Lester BW, Pan TD, Robinson-Bostom L. Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes. J Cutan Pathol 33(10):695-700. October 2006
77. Grobner T. Gadolinium--a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? [Reply to letter] Nephrol Dial Transplant. Epub October 2006
78. Galan A, Cowper SE, Bucala R. Nephrogenic systemic fibrosis (nephrogenic fibrosing dermopathy). Curr Opin Rheumatol. 18(6):614-7. November 2006
79. Thomsen HS, Morcos SK, Dawson P. Is there a causal relation between the administration of gadolinium based contrast media and the development of nephrogenic systemic fibrosis (NSF)? Clin Radiol 61(11):905-6. November 2006
80. Thomsen HS. Nephrogenic systemic fibrosis: a serious late adverse reaction to gadodiamide. Eur Radiol. Epub October 24, 2006
81. High WA, Ayers RA, Chandler J, Zito G, Cowper SE. Gadolinium is detectable within the tissue of patients with nephrogenic systemic fibrosis [pdf, free download]. J Am Acad Dermatol. Epub November 9, 2006
82. Boyd AS, Zic JA, Abraham JL. Gadolinium deposition in nephrogenic fibrosing dermopathy [pdf, free download]. J Am Acad Dermatol. Epub November 13, 2006
83. Dharnidarka VR, Wesson SK, Fennell RS. Gadolinium and nephrogenic fibrosing dermopathy in pediatric patients [Letter]. Pediatr Nephrol. Epub December 2006
84. Aydingoz U. The need for radiologists' awareness of nephrogenic systemic fibrosis [pdf, free download]. Diagn Interven Radiol. 12(4):161-2. December 2006
85. Thomsen HS. European Society of Urogenital Radiology Guidelines on contrast media application. Curr Opin Urol. 17(1):70-6. January 2007
86. Kuo PH, Kanal E, Abu-Alfa AK, Cowper SE. Gadolinium-based MR contrast agents and nephrogenic systemic fibrosis. Radiology. Epub January 9, 2007
87. Solomon GJ, Wu E, Rosen PP. Nephrogenic Systemic Fibrosis mimicking inflammatory breast carcinoma [pdf, free download]. Radiology. Epub January 9, 2007
88. Mitka M. MRI contrast agents may pose risk for patients with kidney disease. JAMA. 297(3):252-3. January 17, 2007
89. Sadowski EA, Bennett LK, Chan MR, Wentland AL, Garrett AL, Garrett RW, Djamali A. Nephrogenic Systemic Fibrosis: Risk Factors and Incidence Estimation. Radiology. Epub January 31, 2007
90. Farlow JT. The enigma of nephrogenic systemic fibrosis. Nephrol Nurs J. 34(1):80-1. Jan-Feb, 2007
91. Broome DR, Girguis MS, Baron PW, Cottrell AC, Kjellin I, Kirk GA. M. Gadodiamide-associated nephrogenic systemic fibrosis: why radiologists should be concerned. Am J Roentgenol. 188(2):586-92. February 2007
92. Cheung PP, Dorai Raj AK. Nephrogenic fibrosing dermopathy: a new clinical entity mimicking scleroderma. Intern Med J 37(2):139-41. February 2007
93. Khurana A, Runge VM, Narayanan M, Greene JF Jr, Nickel AE. Nephrogenic Systemic Fibrosis: A Review of 6 Cases Temporally Related to Gadodiamide Injection (Omniscan). Invest Radiol 42(2):139-45. February 2007
94. Weiss AS, Lucia MS, Teitelbaum I. A case of nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis. [Full Text] Nat Clin Pract Nephrol 3(2):111-5. February 2007
95. High WA, Ayers RA, Cowper SE. Gadolinium is quantifiable within the tissue of patients with nephrogenic systemic fibrosis. [Full Text]J Am Acad Dermatol 56(4):710-2. Epub February 6, 2007
96. Hellman RN. Erythropoietin, gadolinium, and nephrogenic fibrosing dermopathy [Letter] [Full Text]. Ann Intern Med 146(3):229-30. February 6, 2007

97. Leung N, Swaminathan S, Ahmed I, et al. Erythropoietin, gadolinium, and nephrogenic fibrosing dermopathy [Response Letter] [Full Text]. Ann Intern Med 146(3):230. February 6, 2007
98. 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 [Full Text] Clin J Am Soc Nephrol 2:258-63. Epub February 7, 2007
99. Deo A, Fogel M, Cowper SE. Nephrogenic Systemic Fibrosis: A Population Study Examining the Relationship of Disease Development to Gadolinium Exposure [Full Text] Clin J Am Soc Nephrol 2:264-7. Epub February 7, 2007
100. Perazella MA. Nephrogenic Systemic Fibrosis, Kidney Disease, and Gadolinium: Is There a Link? (Editorial) [Full Text] Clin J Am Soc Nephrol 2:200-2. Epub February 7, 2007
101. Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents--St. Louis, Missouri, 2002-2006.[Full Text]. MMWR Morb Mortal Wkly Rep 56(7):137-41. February 23, 2007
102. Hedley AJ, Molan MP, Hare DL, Anavekar NS, Ierino FL. Nephrogenic systemic fibrosis associated with gadolinium-containing contrast media administration in patients with reduced glomerular filtration rate [Letter] [Full Text]. Nephrology 12(1):111. February 2007

103. 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 156(3):473-9. March 2007
104. Chewning RH, Murphy KJ. Gadolinium-based Contrast Media and the Development of Nephrogenic Systemic Fibrosis in Patients with Renal Insufficiency. [Full Text] J Vasc Interv Radiol. 18(3):331-3. March 2007
105. Krous H, Breisch E, Chadwick A, Pinckney L, Malicki D, Benador N. Nephrogenic Systemic Fibrosis with Multiorgan Involvement in a Teenage Male after Lymphoma, Ewing Sarcoma, End Stage Renal Disease, and Hemodialysis. Pediatr Dev Pathol. Epub. March 22, 2007

* [IMPORTANT NOTE] Contrary to the implication of the terminology: "Dialysis Associated Systemic Fibrosis", NSF is not caused by dialysis. This has been well established for years, and it is an unfortunate choice of words in this suggestion of a name change for NSF.

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