Erdheim-Chester disease: CT findings of thoracic involvement.

Details

Serval ID
serval:BIB_9B7A1BFC5BB0
Type
Article: article from journal or magazin.
Collection
Publications
Title
Erdheim-Chester disease: CT findings of thoracic involvement.
Journal
European Radiology
Author(s)
Brun A.L., Touitou-Gottenberg D., Haroche J., Toledano D., Cluzel P., Beigelman-Aubry C., Piette J.C., Amoura Z., Grenier P.A.
ISSN
1432-1084 (Electronic)
ISSN-L
0938-7994
Publication state
Published
Issued date
2010
Volume
20
Number
11
Pages
2579-2587
Language
english
Abstract
OBJECTIVE: To retrospectively assess the association of mediastinal, cardiovascular and pleuropulmonary findings on chest CT of 40 patients with immunohistochemically and histologically proven Erdheim-Chester disease (ECD). METHODS: The multidetector chest CT images of 40 ECD patients were reviewed in consensus by chest and cardiovascular radiologists. RESULTS: Thirty-four (85%) patients had periaortic infiltration that extended around the aortic branches of 29 (73%). Perivascular infiltration extended into the cardiac sulci in 22 (55%) (p < 0.005). Infiltration involved the right atrium wall in 12 patients, associated with severe narrowing of the atrial lumen in 8. Pericardial effusion and/or thickening were observed in 24 (60%) patients. Lung involvement, seen in 22 (55%) patients, was associated with mediastinal infiltration (20; p < 0.005) and pleural thickening or effusion (16; p = 0.001); it consisted of smooth interlobular septa (21), subpleural thickening (13), poorly defined centrilobular nodular opacities (9), ground-glass opacities (8) and/or lung cysts (5). CONCLUSION: The detailed description of thoracic ECD involvement seen in these patients showed that infiltration into the mediastinal spaces including the pericardium, coronary sulci and right atrium is frequently associated with pleural and interstitial lung diseases.
Keywords
Adult, Aged, Erdheim-Chester Disease/complications, Erdheim-Chester Disease/radiography, Female, Heart Diseases/complications, Heart Diseases/radiography, Humans, Lung Diseases/complications, Lung Diseases/radiography, Male, Mediastinal Diseases/complications, Mediastinal Diseases/radiography, Middle Aged, Pleural Diseases/complications, Pleural Diseases/radiography, Radiography, Thoracic, Thoracic Diseases/complications, Thoracic Diseases/radiography, Tomography, X-Ray Computed, Young Adult
Pubmed
Web of science
Create date
31/08/2011 12:46
Last modification date
20/08/2019 15:02
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