NEPHROGENIC SYSTEMIC FIBROSIS

Description

  • It is rare condition that occurs mainly in people with advanced kidney failure with or without dialysis

Symptoms

  • Swelling and tightening of the skin
  • Thickening and hardening of the skin
  • Skin that may feel “woody”
  • excess pigmentation
  • Burning, itching or severe sharp pains in areas of involvement
  • Muscle weakness
  • Bone pain
  • Blood clots

Causes

  • Exposure to gadolinium-containing contrast agents  during MRI
  • Use of high-dose erythropoietin (EPO)
  • Recent vascular surgery
  • Blood-clotting problems
  • Severe infection
  • Have moderate to severe kidney disease
  • kidney transplant
  • Receiving hemodialysis or peritoneal dialysis
  • Acute kidney injury

Diagnostic Tests

  • Physical examination
  • Skin biopsy

Treatment

  • Hemodialysis
  • Physical therapy
  • Kidney transplant
  • Extracorporeal photopheresis
  • Plasmapheresis
  • Medicines:
  • Pentoxifylline (Pentoxil)
  • Sodium thiosulfate

Lifestyle Management

  • Avoid gadolinium-containing contrast agents
  • Healthy diet