Erdheim-Chester Disease - Multisystemic Radiological Manifestations

Abstract

Erdheim-Chester disease (ECD) is a rare, multisystem disorder with a constellation of clinical and radiological findings. We present a case of ECD who came with acute obstructive uropathy and chronic bone pain. Radiological work up in this patient led to a host of findings in various systems, the correlation of which led to narrowing of the differential diagnoses. 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).