Imaging Pearls ❯ Kidney ❯ Erdheim-Chester Disease
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- Erdheim–Chester disease: Renal Findings
- enlarged kidneys
- perirenal infiltrations shown as characteristic “hairy kidney” (70%)
- Renal sinus involvement
- Thickening of the ureters
- Soft tissue thickening of the renal arteries - “In summary, urinary involvement in ECD was mostly bilateral and symmetric. Early identification of the perirenal hairy kidney sign is critical for accurate diagnosis and timely treatment. Understanding of the CT findings and progression as well as correct diagnosis based on radiological findings, have significant implications for guiding ECD treatment.”
Urinary involvement in Erdheim–Chester disease: computed tomography imaging findings
Zhe Wu et al.
Abdominal Radiology (2021) 46:4324–4331 - Erdheim Chester Disease: Renal Changes
- Perirenal fat effaced or infiltrated by soft tissue
- Usually bilateral and symmetric
- Infiltration of para-aortic regions is also common - Erdheim Chester Disease: Vascular Changes
- Periaortic infiltration which is usually circumferential and nonocclusive
- May involve aorta from root thru iliac vessels
- May involve the renal arteries - Erdheim Chester Disease: Skeletal Changes
- Bilateral symmetric osteosclerosis of metaphyses and diaphyses especially in long bone.