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
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