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Abstract Number: 225

Cardiovascular Involvement In Erdheim-Chester Disease: A Magnetic Resonance Imaging Study On Seven Patients

Enrica Rossi1, Lorenzo Buttarelli1, Augusto Vaglio2, Davide Gianfreda3, Chiara Martini1 and Massimo De Filippo1, 1Radiology, University of Parma, Parma, Italy, 2Unit of Nephrology, University Hospital of Parma, Parma, Italy, 3University of Parma, Parma, Italy

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Erdheim-Chester disease, MRI and cardiovascular disease

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Session Information

Title: Imaging of Rheumatic Diseases I: Imaging in Gout, Pediatric, Soft and Connective Tissue Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Erdheim-Chester Disease (ECD) is a rare non-Langherans form of histiocytosis, characterized by xanthomatous or xanthogranulomatous infiltration of tissues by foamy histiocytes, surrounded by fibrosis. ECD is a multisystemic disease and  its clinical course depends mostly on cardiovascular manifestations, that are responsible for poor prognosis and death. In order to assess the cardiovascular extent of the disease, we studied 7 consecutive cases with biopsy-proven ECD by magnetic resonance imaging (MRI).

Methods:

The patients underwent cardiac and thoracic aorta MRI. Images were acquired with a 1,5T RM, using T2W with and without fat suppression, long and short axis cine B-TFE and late gadolinium enhancement sequences evaluated by dedicated software.

Results:

Four patients (57%) showed abnormal heart imaging. Four patients had myocardium involvement with typical pseudo-mass aspects in the anterior and lateral walls of right atrium. In three cases it extended to the right atrioventricular sulcus, sheathing the right coronary artery; only in one case both coronary arteries were surrounded by periarterial infiltration. None of the cases showed signs of coronary stenosis. One case also presented atrial septal thickening. In three cases imaging studies recognized pericardial effusion; just in one case it was massive (50mm), even if without signs of cardiac tamponade. The pericardium facing the right ventriculum was thickened in two cases.

Imaging showed a mild cardiomegaly in one patient, but no atrial enlargement was detected. In no case we recognized cardiac insufficiency or cardiac signs of systemic hypertension. No patients had valvular defects.

The angiography assessment suggested the presence of periaortic fibrosis with “coated aspect” in two cases. In these, epiaortic vessels were surrounded by irregular, non stenosing fibrosis.

Conclusion:

Our study confirms the frequent cardiovascular involvement in ECD. A systematic cardiac and aortic evaluation by MRI would be indicated in patients with ECD.


Disclosure:

E. Rossi,
None;

L. Buttarelli,
None;

A. Vaglio,
None;

D. Gianfreda,
None;

C. Martini,
None;

M. De Filippo,
None.

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