Advertisement

Nephrogenic Systemic Fibrosis

Chapter

Abstract

Nephrogenic systemic fibrosis (NSF) is a debilitating fibrosing disorder that has been observed almost exclusively among patients with compromised renal function following exposure to gadolinium-containing contrast agents. It presents with brawny hyperpigmentation and tethering of skin, predominantly on the extremities, and fixed flexion contractures of the knees, ankles, elbows, and fingers. Yellow scleral plaques with capillary dilatation may be evident on the eyes and visceral involvement can cause significant morbidity and mortality. A deep skin biopsy demonstrating increased numbers of spindle-shaped fibroblast-like cells with dual CD34- and procollagen I-positive immunohistochemical staining in the dermis and thickened collagen bundles separated by adjacent clefts confirms the diagnosis of nephrogenic systemic fibrosis in a patient with characteristic clinical features. Treatment with tyrosine kinase inhibitors may reverse existing fibrosis and prevent progression of disease.

Keywords

Chronic Kidney Disease Nephrogenic Systemic Fibrosis Osseous Metaplasia Nephrogenic Fibrosing Dermopathy Fibrosing Disorder 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE. Scleromyxoedema-like cutaneous diseases in renal-dialysis patients. Lancet. 2000;356:1000–1.PubMedCrossRefGoogle Scholar
  2. 2.
    Girardi M, Kay J, Elston DM, LeBoit PE, Ali Abu-Alfa A, Cowper SE. Nephrogenic systemic fibrosis: clinicopathological definition and work-up recommendations. J Am Acad Dermatol. 2011;65:1095–1106.e7.PubMedCrossRefGoogle Scholar
  3. 3.
    Grobner T. Gadolinium–a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis? Nephrol Dial Transplant. 2006;21:1104–8.PubMedCrossRefGoogle Scholar
  4. 4.
    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;56:3433–41.PubMedCrossRefGoogle Scholar
  5. 5.
    Abujudeh HH, Kaewlai R, Kagan A, Chibnik LB, Nazarian RM, High WA, Kay J. Nephrogenic systemic fibrosis after gadopentetate dimeglumine exposure: case series of 36 patients. Radiology. 2009;253:81–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Cowper SE, Su LD, Bhawan J, Robin HS, LeBoit PE. Nephrogenic fibrosing dermopathy. Am J Dermatopathol. 2001;23:383–93.PubMedCrossRefGoogle Scholar
  7. 7.
    Koreishi AF, Nazarian RM, Saenz AJ, Klepeis VE, McDonald AG, Farris AB, Colvin RB, Duncan LM, Mandal RV, Kay J. Nephrogenic systemic fibrosis: a pathologic study of autopsy cases. Arch Pathol Lab Med. 2009;133:1943–8.PubMedGoogle Scholar
  8. 8.
    Kay J, Bazari H, Avery LL, Koreishi AF. Case records of the Massachusetts General Hospital. Case 6–2008. A 46-year-old woman with renal failure and stiffness of the joints and skin. N Engl J Med. 2008;358:827–38.PubMedCrossRefGoogle Scholar
  9. 9.
    Nazarian RM, Mandal RV, Kagan A, Kay J, Duncan LM. Quantitative assessment of dermal cellularity in nephrogenic systemic fibrosis: a diagnostic aid. J Am Acad Dermatol. 2011;64:741–7.PubMedCrossRefGoogle Scholar
  10. 10.
    High WA, Ayers RA, Cowper SE. Gadolinium is quantifiable within the tissue of patients with nephrogenic systemic fibrosis. J Am Acad Dermatol. 2007;56:710–2.PubMedCrossRefGoogle Scholar
  11. 11.
    Wang Y, Narin O, Alkasab TK, Nazarian RM, Kaewlai R, Kay J, Abujudeh HH. Incidence of nephrogenic systemic fibrosis following adoption of a restrictive gadolinium policy. Radiology. 2011;260:105–11.PubMedCrossRefGoogle Scholar
  12. 12.
    Kay J, High WA. Imatinib mesylate treatment of nephrogenic systemic fibrosis. Arthritis Rheum. 2008;58:2543–8.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2014

Authors and Affiliations

  1. 1.Rheumatology Division, Department of MedicineUniversity of Massachusetts School of MedicineWorcesterUSA
  2. 2.Dermatopathology Unit, Department of PathologyMassachusetts General Hospital and Harvard Medical SchoolBostonUSA

Personalised recommendations