Erdheim-Chester Disease - Multisystemic Radiological Manifestations

Abstract

Rashmi Mysore Nagaraju1, Bhimarao2

A 44-year-old male presented with right loin pain and nausea for 1-day duration.
A provisional diagnosis of right ureteric colic was made. Routine lab investigations
revealed normal total counts and urine analysis. Additional laboratory
investigations revealed a raised erythrocyte sedimentation rate (ESR) and Creactive
protein (CRP).
Radiological Investigations
1. Patient was referred for ultrasound of abdomen which revealed mild to
moderate right hydronephrosis with a suspicious hypoechoic area (~ 0.7 x 1.2
cm) in the renal pelvis region raising possibility of a lesion.
2. Contrast computed tomography (CT) study of abdomen was performed which
revealed enhancing soft tissue infiltration (+30 to +45 HU) around bilateral
kidneys (giving ‘hairy kidney appearance’) (Figures 1, 2 & 3) and adrenal glands
with infiltration to renal sinus and constriction of bilateral renal pelves resulting
in moderate right and mild left hydronephrosis. Thin streak of excreted contrast
was traversing the narrowed segment of renal pelves (Figures 4 & 5).
3. Soft tissue infiltration covering the visualized thoracic and abdominal aorta
(giving ‘coated aorta appearance’) (Figures 6 & 7), extending below renal
arteries to aortic bifurcation and proximal bilateral common iliac arteries.
4. The adrenal glands were not separately visualized from the above mentioned
perinephric soft tissue.
5. Both ureters were normal in course and caliber. No evidence of ureteric
calculus.
6. No significant retroperitoneal lymphadenopathy.
7. Few tiny centriacinar nodules with patchy ground glass opacities in postero
basal segment of both lungs (left > right) (Figure 8).

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