Erdheim-Chester disease (pulmonary manifestations)

Pulmonary manifestations of Erdheim-Chester disease are uncommon.

Epidemiology

The lungs are affected in ~25% (range 20-35%) of cases .

Radiographic features

HRCT chest

Described findings include

• symmetric reticular interstitial opacities
• smooth interlobular septal thickening and fissural thickening
• diffuse multifocal regions of ground-glass attenuation
• centrilobular nodular opacities
• pleural effusions

Look also extra-pulmonary features such as

• peri-aortic infiltration: may extend to the ascending aorta, coronary arteries, and aortic branches
• infiltration of the pulmonary arteries, superior vena cava, pericardium, and myocardium

Treatment and prognosis

Pulmonary involvement is generally an indicator or poor prognosis .