Revista Española de Cardiología (English Edition) Revista Española de Cardiología (English Edition)
Rev Esp Cardiol. 2016;69:72 - Vol. 69 Num.01 DOI: 10.1016/j.rec.2015.04.004

Erdheim-Chester Syndrome With Periaortitis

Daniel Rodríguez Sánchez a,, Daniel Saura Espín b, Águeda Bas Bernal c

a Departamento de Radiodiagnóstico, Hospital Clínico Universitario Virgen de la Arrixaca, El Palmar, Murcia, Spain
b Departamento de Cardiología, Hospital Clínico Universitario Virgen de la Arrixaca, El Palmar, Murcia, Spain
c Departamento de Anatomía Patológica, Hospital Clínico Universitario Virgen de la Arrixaca, El Palmar, Murcia, Spain

Article

Aortitis and periaortitis encompass a number of diseases, whose common feature is inflammation of the aortic wall. Their clinical presentation is variable, with nonspecific symptoms such as abdominal pain, fever, and weight loss. For this reason, imaging techniques are of great value.

The patient was a 47-year-old man with suspected acute aortic syndrome, in whom computed tomography angiography revealed circumferential thickening with thoracoabdominal periaortic soft tissue density, irregularity of the aortic lumen (Figure 1, arrows), and ostial stenosis of the left subclavian artery, celiac trunk, and superior mesenteric artery (Figure 1, arrows). The kidneys showed bilateral, symmetrical infiltration of the perirenal tissue with dilation of the renal collecting system (Figure 2, arrows). Perirenal biopsy showed extensive fibrosis with foamy macrophages (Figure 3, arrow) and positive staining for CD68 (Figure 3). The findings were compatible with Erdheim-Chester disease and treatment with pegylated interferon alpha was begun.

Figure 1.

Figure 2.

Figure 3.

This disease is a rare form of nonhereditary histiocytosis that invariably affects the long bones. Retroperitoneal, cardiovascular, or nervous system involvement is detected in 50% of affected individuals. The disease is characterized by infiltration of the connective and perivascular tissue by foamy histiocytes that stain positively for CD68, CD163, and factor XIIIa.

Aortic involvement is common and consists of circumferential periadventitial infiltration, with possible ostial stenosis in the visceral branches. Cardiac involvement can be found in the coronary arteries, pericardium, or atrial wall. The prognosis is determined by the cardiovascular complications.

Corresponding author: danielrs77@hotmail.com

1885-5857/© 2016 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved

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