Erdheim-Chester disease: CT findings of thoracic involvement.
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).


CONCLUSIONS
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.
Brun
Anne-Laure
AL
Radiologie, Hôpital Pitié-Salpêtrière, Assistance Publique-Hôpitaux de Paris, Université Pierre et Marie Curie, Paris VI, Paris, France.
Touitou-Gottenberg
Diane
D
Haroche
Julien
J
Toledano
Dan
D
Cluzel
Philippe
P
Beigelman-Aubry
Catherine
C
Piette
Jean-Charles
JC
Amoura
Zahir
Z
Grenier
Philippe A
PA
D000328:Adult; D000368:Aged; D031249:Erdheim-Chester Disease / Q000150:complications / Q000000981:diagnostic imaging*; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000000981:diagnostic imaging; D006801:Humans; D008171:Lung Diseases / Q000150:complications / Q000000981:diagnostic imaging; D008297:Male; D008477:Mediastinal Diseases / Q000150:complications / Q000000981:diagnostic imaging; D008875:Middle Aged; D010995:Pleural Diseases / Q000150:complications / Q000000981:diagnostic imaging; D013902:Radiography, Thoracic; D013896:Thoracic Diseases / Q000150:complications / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed; D055815:Young Adult