Nephrogenic Systemic Fibrosis

  • Mechanism
    • Occurs in patients with renal disease exposed to gadolinium
    • Teoria: a acidose estimula a libertação de compostos tóxicos do gadolíneo
  • Clinical presentation
    • Ill-defined, thick, indurated plaques
    • Distributed symmetrically on the extremities and trunk
    • Plaques are erythematous to hyperpigmented
    • Can have irregular advancing edge with ameboid appearence
    • Confluent involvement on the extremities often results in joint contractures, hence sclerodermoid disorder
    • Associated with pain and loss of mobility due to the contractures
  • Extracutaneous manifestations
    • Yellow scleral plaques
    • Systemic fibrosis affecting the heart, lungs and skeletal muscles
  • Histology
    • Fibrosis → + fibroblasts with normal collagen (≠sclerosis)
      • Abnormal fibrous dermal (and sometimes subcutaneous) tissue with an increased number of fibroblasts and increased, but rather unremarkable, collagen
  • Treatment
    • Most helpful: restore renal function
    • Avoid use of some more risky gadulineum products if GFR<30
    • Renal transplantation
    • UVA1 phtotherapy
    • Phtopheresis
    • IVIg
    • Photodynamic therapy
    • Imatinib mesylate