대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Erdheim-Chester disease

Radiologic Findings

Fig. 1. Chest radiograph showing increased peribronchovascular interstitial thickening with septal line thickening in both lungs, and cardiomegaly.
Figs. 2 and 3. Chest computed tomography (CT) scans showing smooth interlobular thickening mainly in both upper lobes.
Fig. 4. Chest CT scan showing diffuse infiltrative wall thickening in the right atrium.
Fig. 5. Chest CT scan showing mild perivascular soft tissue encircling the aorta.
Fig. 6. Fluorodeoxyglucose (FDG) positron emission tomography image showing avidly increased uptake of FDG in the infiltrative lesion in the right atrium.

Brief Review

Erdheim-Chester disease (ECD) is a rare sporadic non-Langerhans cell histiocytic (LCH) proliferative disorder with systemic predilection. It usually affects adults in the fifth to seventh decades of life, with a slight male predilection. It has nonspecific clinical manifestations depending on the site of involvement. Constitutional symptoms, such as fever, weight loss, and night sweats, are common. Overall, the skeletal system is the most common site of involvement, leading to bone pain, which is the presenting manifestation in nearly 96% of cases. The most common extraosseous manifestations are central diabetes insipidus and exophthalmos.
The clinical, radiologic, and light microscopic findings may be indistinguishable from those of LCH. It is often suspected in the presence of characteristic radiologic findings and is subsequently confirmed by histopathology.
Histopathologically, ECD is characterized by tissue infiltration by lipid-laden histiocytes. The demonstration of CD68 positivity in xanthogranulomatous histiocytic infiltrates with a simultaneous lack of CD1a and Birbeck granules is confirmatory, thereby aiding in differentiating ECD from LCH.
Imaging plays a key role in the diagnosis, management, and follow-up. Plain radiography and technetium-99m bone scintigraphy are useful in determining skeletal involvement. Computed tomography (CT) or magnetic resonance imaging are useful for the evaluation of thoracoabdominal and central nervous system (CNS) involvement. Fluorodeoxyglucose-positron emission tomography/CT is invaluable for the in vivo determination of disease activity during follow-up.
The lung parenchyma and pleura are affected in approximately 40%

Please refer to

Case 744,

References

1. Kumar, Pawan, et al. "Imaging findings in Erdheim-Chester disease: what every radiologist needs to know." Polish journal of radiology 83 (2018): e54.
2. Wittenberg, Keith H., Stephen J. Swensen, and Jeffrey L. Myers. "Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings." American Journal of Roentgenology 174.5 (2000): 1327-1331.

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