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Kidney CT Appearances

 

 


Erdheim-Chester Disease CT Findings

  • Enlarged kidneys
  • Perirenal infiltrations shown as characteristic “hairy kidney” in 70% of cases
  • Renal sinus involvement
  • Thickening of the ureters
  • Soft tissue thickening of the renal arteries
  • Perirenal fat effaced or infiltrated by soft tissue
  • Usually bilateral and symmetric
  • Infiltration of para-aortic regions is also common

Erdheim-Chester CT Findings

Other Information About Erdheim-Chester Disease

Etiology:

  • Unknown

Epidemiology:

  • Most common in 40-60 year olds
  • Range age at diagnosis is 7-84
  • More common in males

Presentation:

  • Bone pain, typically in the legs and arms
  • Diabetes insipidus
  • Dysuria
  • Night sweats
  • Weight loss
  • Xanthelasma
  • Bulging, red eyes

Prognosis:

  • Currently 5-year survival rate is around 80%

Related Pearls: Erdheim-Chester Disease

Related Lectures:
CT of the Perirenal Space: Differential Diagnosis
CT Evaluation of Vasculitis: Key Imaging Findings - Part 2

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