Session Type: Abstract Submissions (ACR)
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).
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.
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.
Our study confirms the frequent cardiovascular involvement in ECD. A systematic cardiac and aortic evaluation by MRI would be indicated in patients with ECD.
M. De Filippo,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cardiovascular-involvement-in-erdheim-chester-disease-a-magnetic-resonance-imaging-study-on-seven-patients/