CTICasesErdheim-Chester Disease
Imaging findings
Chest and abdominal CT shows soft tissue encasing the thoracic and abdominal aorta (coated aorta), infiltrating the perinephric spaces (hairy kidneys), and thickening the posterior wall of the right atrium. PET shows intense FDG avidity of these lesions. Cardiac MRI confirms right atrial infiltration.
Key takeaways
Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis. Classic features include a 'coated aorta', 'hairy kidneys' due to retroperitoneal fibrosis, and infiltration of the right atrium/interatrial septum.
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