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Pulmonary Involvement with Erdheim-Chester Disease

Radiographic and CT Findings

¹ Department of Diagnostic Radiology, Mayo Clinic, 200 First St. S.W., Rochester, MN 55905.
² Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905.

View larger version (151K): [in a new window]   Fig. 1A. —51-year-old man with Erdheim-Chester disease. Posteroanterior (A) and lateral (B) chest radiographs reveal diffuse reticular interstitial process throughout both lungs with associated fissural thickening (arrows in B).

View larger version (133K): [in a new window]   Fig. 1B. —51-year-old man with Erdheim-Chester disease. Posteroanterior (A) and lateral (B) chest radiographs reveal diffuse reticular interstitial process throughout both lungs with associated fissural thickening (arrows in B).

View larger version (158K): [in a new window]   Fig. 1C. —51-year-old man with Erdheim-Chester disease. Magnification view of right lung base from subsequent radiograph shows prominent Kerley B lines (arrowheads), indicating interlobular septal thickening.

View larger version (127K): [in a new window]   Fig. 2A. —70-year-old man with Erdheim-Chester disease. High-resolution chest CT scan (1-mm collimation) shows diffuse smooth interlobular septal thickening (arrowheads) and patchy areas of ground-glass attenuation (arrows).

View larger version (202K): [in a new window]   Fig. 2B. —70-year-old man with Erdheim-Chester disease. Soft-tissue high-resolution CT scan through upper abdomen shows soft-tissue stranding that surrounds both kidneys (arrows). Biopsy specimen revealed histiocytic infiltration with non—Langerhans' cells, characteristic of Erdheim-Chester disease.

View larger version (117K): [in a new window]   Fig. 3A. —37-year-old woman with dyspnea. High-resolution CT scan through lung bases shows prominent centrilobular nodular opacities (straight solid arrows), interlobular septal thickening (curved arrows), and fissural thickening (open arrows) in both lung bases.

View larger version (126K): [in a new window]   Fig. 3B. —37-year-old woman with dyspnea. Low-magnification photomicrograph shows visceral pleural (straight arrows) and interlobular septal thickening (curved arrows). Pleurae and interlobular septa are thickened by combination of mixed inflammatory opacity and fibrosis with relative sparing of alveolar septa.

View larger version (175K): [in a new window]   Fig. 4. —43-year-old woman with Erdheim-Chester disease and dyspnea. Low-magnification photomicrograph reveals dramatic lymphangitic distribution of abnormalities in lung biopsy. Visceral pleurae (straight arrows), interlobular septae (curved arrows), and bronchovascular bundles (open arrows) are expanded by combination of inflammation and fibrosis.

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