Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings

We determined the chest radiographic and CT findings of Erdheim-Chester disease with pulmonary involvement.

We retrospectively reviewed the radiologic images of 15 patients with biopsy-proven Erdheim-Chester disease. Nine patients had chest radiographic images and CT scans that were available for review. Six men and three women were studied (age range, 25-70 years; mean age, 56 years). Two radiologists interpreted all images by consensus. Lung parenchyma was assessed for the type and distribution of disease. Bronchi, pleurae, hila, and mediastinal and extrathoracic structures were evaluated for abnormalities. Pathologic specimens from all patients were reviewed and correlated with radiologic findings.

Eight of nine patients had thoracic images with abnormal findings. The most common radiographic pattern was reticular interstitial opacities with fissural and interlobular septal thickening. CT revealed regions of ground-glass attenuation and centrilobular nodular opacities. Typically, extrapulmonary findings included pleural effusions (6/8 patients), pericardial fluid or thickening (4/8), and extrathoracic infiltrative soft-tissue masses (4/8).

The most common findings of Erdheim-Chester disease with pulmonary involvement include an interstitial process characterized by smooth interlobular septal thickening and centrilobular nodular opacities, fissural thickening, and pleural effusions. On CT, six of nine patients had pericardial fluid and thickening or extrathoracic soft-tissue masses. Such findings are characteristic of Erdheim-Chester disease with pulmonary involvement. Definitive diagnosis requires correlating skeletal findings and lung biopsy findings.