Subscribe to RSS

DOI: 10.12945/j.aorta.2013.13-023
Imaging Assessment of Periaortic Inflammation in Erdheim-Chester Disease
Publication History
09 April 2013
25 May 2013
Publication Date:
28 September 2018 (online)

Abstract
Reaching etiologic diagnoses for retroperitoneal fibrosis may be challenging. We report the case of a 75-year old male with history of ruptured abdominal aortic aneurysm and subsequent retroperitoneal fibrosis who developed four years later a soft tissue infiltration surrounding the ascending thoracic aorta. Thanks to his medical records and multimodality imaging assessment, the patient escaped an open-chest biopsy through histolgical reassessment of the abdominal periaortic samples that allowed the definitive diagnosis of Erdheim-Chester disease, a rare non-Langerhans histiocytosis.
-
References
- 1 Dion E, Graef C, Haroche J, Renard-Penna R, Cluzel P, Wechsler B. , et al. Imaging of thoracoabdominal involvement in Erdheim-Chester disease. AJR Am J Roentgenol 2004; 183: 1253-1260 . 10.2214/ajr.183.5.1831253
- 2 Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367: 241-251 . 10.1016/S0140-6736(06)68035-5
- 3 Jennette JC, Falk RJ. The role of pathology in the diagnosis of systemic vasculitis. Clin Exp Rheumatol 2007; 25: S52-S56