Home

Current Issue

Archives

Guidelines for Authors

Classified Ads

Subscriptions

Contact Info

Email Alert Service

Links

“Coated Aorta”: A New Sign of Erdheim-Chester Disease

JACQUES SERRATRICE, BRIGITTE GRANEL, CHRISTINE DE ROUX, JEAN-FRANÇOIS PELLISSIER, LAURE SWIADER, JEAN-MICHEL BARTOLI, PATRICK DISDIER, and PIERRE-JEAN WEILLER

ABSTRACT.

Erdheim-Chester disease is a rare, non-Langerhans cell form of histiocytosis characterized by osteosclerosis of the metaphyseal regions of long bones, diabetes insipidus, proptosis, and retroperitoneal fibrosis. The latter usually involves the perirenal area and leads to hydronephrosis. Periaortic fibrosis is less frequent. We describe 3 unusual cases of Erdheim-Chester disease with periaortic fibrosis involving the whole aorta and leading to a “coated aorta” appearance on computed tomography scans. Faced with such a singular “coated aorta,” bone scintigraphy can be very helpful when searching for Erdheim-Chester disease. (J Rheumatol 2000;27:1550–3)

Key Indexing Terms:

ERDHEIM-CHESTER DISEASE
PERIAORTITIS
AORTA