Erdheim-Chester disease: CT findings of thoracic involvement.

Objective: To retrospectively assess the association of mediastinal, cardiovascular and pleuropulmonary findings on chest CT of 40 patients with immunohistochemically and histologically proven Erdheim-Chester disease (ECD).

Methods: The multidetector chest CT images of 40 ECD patients were reviewed in consensus by chest and cardiovascular radiologists.

Results: Thirty-four (85%) patients had periaortic infiltration that extended around the aortic branches of 29 (73%).