PMC4840400_01
Unenhanced thoracic CT prior to therapy initiation displayed in soft tissue (A) and lung (B) windows shows bilateral pleural effusions ( ), a moderate-sized pericardial effusion (*), and basal interlobular septal thickening (arrowheads). Enhanced thoracic CT displayed in soft tissue (C and lung (D) windows, performed approximately one year following initiation of corticosteroid therapy, shows reduction in bilateral pleural effusions ( ) and pericardial effusion (*), but with increasing interlobular septal thickening (arrowheads). Perivascular soft tissue infiltration surrounding the descending thoracic aorta (arrows) is evident.
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A 61-year-old previously healthy male with a history of progressive fatigue, lower extremity edema, and dyspnea for 4 months was hospitalized at an outside facility and diagnosed with a pericardial effusion and bilateral pleural effusions (Fig. 1A and B). Repeat thoracic computed tomography (CT) (Fig. 1C and D) showed bilateral pleural effusions, pericardial effusions, and diffuse interlobular septal thickening.
Repeat enhanced thoracic CT prior to initiation of cladribine therapy, but following PEG interferon treatment, displayed in soft tissue (A) and lung (B) windows shows resolution of pericardial fluid but with development of pericardial and cardiac soft tissue infiltration (*); similarly, pleural liquid has resolved, but extensive pleural thickening ( ) has developed. Interlobular septal thickening (arrowheads) with patchy bilateral increased lung opacity and periaortic soft tissue infiltration (arrows) persist. Enhanced thoracic CT displayed in soft tissue (C) and lung (D) windows 4 months following completion of 3 cycles of cladribine therapy shows persistent but decreased bilateral medial basal pleural thickening ( ) with persistent but slightly less pronounced bilateral interlobular septal thickening (arrowheads) and overall improved in bilateral lung opacity compared to (B).
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While the abnormalities on thoracic CT remained stable (Fig. 2A and B), PFTs revealed worsening restriction and worsening of diffusion capacity (FVC decreased from 46% to 34%, and DCLO 55% to 36%). PFTs and findings on CT chest also improved. (Fig. 2C and D).
Wedge biopsy of lung, including pleura. There is both pleural and interlobular septal thickening (left panel, hematoxylin-eosin, x4), as a result of an accumulation of foamy macrophages (right panel, hematoxylin-eosin, x400). These macrophages expressed CD68, and Factor XIIIa, but not CD1a, as is characteristic of Erdheim-Chester disease.
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Lung, pericardial, and pleural biopsies were consistent with Erdheim-Chester disease (ECD) (Fig. 3, Fig. 4).