Erdheim-Chester disease is a rare, idiopathic, multisystemic non-Langerhans cell histiocytosis. Little is known about the imaging features. Herein, we report a very uncommon case of Erdheim-Chester disease in a 54-year-old woman with multisystem involvement including cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration), orbit and pituitary. Multimodal imaging modalities, including computed tomography, magnetic resonance imaging, echocardiography, and bone scintigraphy were used to comprehensively evaluate different organs involvement. Finally, myocardial biopsy results indicated Erdheim-Chester disease. Electrocardiography showed sick sinus syndrome and slowest heart rate of 20 beats/min. The patient underwent permanent pacemaker implantation and had initial treatment with interferon. There were no remarkable changes in right atrial lesion during 9-month follow-up period. Erdheim-Chester disease was a rare entity with a dismal prognosis, especially when there were cardiac and neurological involvement. The present case report aimed to described and analyzed radiological findings of multiple organs involvement of Erdheim-Chester disease with multimodal imaging retrospectively, and being familiar with the imaging features of Erdheim-Chester disease might help prompt and correct diagnosis of this disease in the future.
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