"Coated aorta": a new sign of Erdheim-Chester disease

J Rheumatol. 2000 Jun;27(6):1550-3.


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.

Publication types

  • Case Reports

MeSH terms

  • Aortitis / diagnostic imaging*
  • Aortitis / pathology
  • Bone and Bones / diagnostic imaging
  • Diphosphonates
  • Fatal Outcome
  • Female
  • Fibrosis
  • Histiocytosis / diagnostic imaging*
  • Histiocytosis / pathology
  • Humans
  • Male
  • Middle Aged
  • Radionuclide Imaging
  • Radiopharmaceuticals
  • Technetium Compounds
  • Tomography, X-Ray Computed*


  • Diphosphonates
  • Radiopharmaceuticals
  • Technetium Compounds
  • technetium Tc 99m diphosphonate