Document Detail


Erdheim-Chester disease: CT findings of thoracic involvement.
MedLine Citation:
PMID:  20563815     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Anne-Laure Brun; Diane Touitou-Gottenberg; Julien Haroche; Dan Toledano; Philippe Cluzel; Catherine Beigelman-Aubry; Jean-Charles Piette; Zahir Amoura; Philippe A Grenier
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Publication Detail:
Type:  Journal Article     Date:  2010-06-20
Journal Detail:
Title:  European radiology     Volume:  20     ISSN:  1432-1084     ISO Abbreviation:  Eur Radiol     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-10-04     Completed Date:  2011-01-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9114774     Medline TA:  Eur Radiol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  2579-87     Citation Subset:  IM    
Affiliation:
Radiologie, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Université Pierre et Marie Curie, Paris VI, Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Erdheim-Chester Disease / complications,  radiography*
Female
Heart Diseases / complications,  radiography
Humans
Lung Diseases / complications,  radiography
Male
Mediastinal Diseases / complications,  radiography
Middle Aged
Pleural Diseases / complications,  radiography
Radiography, Thoracic
Thoracic Diseases / complications,  radiography*
Tomography, X-Ray Computed*
Young Adult

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