Amyloidosis and Lymphoproliferative disease in Sjögren syndrome: Thin-section computed tomography findings and histopathologic comparisons

Objective: To describe the thin-section computed tomography (CT) findings of Sjögren syndrome accompanying pulmonary amyloidosis and lymphoproliferative disease and to compare these with histopathologic findings. Subjects and Methods: The thin-section CT findings of 5 women (age range: 42-59 years, mean age = 50 years) with primary Sjögren syndrome accompanying pulmonary amyloidosis and lymphoproliferative disease were reviewed retrospectively by 2 chest radiologists, and decisions on findings were reached by consensus. The pathologic specimens of parenchymal lesions (nodules, dense consolidation, and cystic lesion at CT) obtained using video-assisted thoracoscopic surgery were studied to compare with the thin-section CT findings. Results: Nodules, observed in all 5 patients, were variable in size and ranged from 3 to 24 mm (mean = 9.9 mm) in diameter, with lobulated or irregular margins. Nodular calcifications were present in 3 patients. Cysts, which also were observed in all patients, ranged from 4 to 45 mm (mean = 18.6 mm) in diameter, with a thin (1-2 mm) or no visible wall. Multiple cysts were observed, especially in the distal portion of narrowed bronchioles. Nodules and cysts showed a random distribution. Mild bronchial wall thickening with bronchial dilatation was seen in all patients, ground-glass opacities were seen in 3, and consolidation was seen in 1. Nodules, consolidation, and bronchial wall thickening at CT were caused histopathologically by the interstitial and peribronchiolar deposition of mixed amyloid and lymphoproliferative cells. Cysts lined with respiratory epithelium contained amyloid deposition and lymphoproliferative cells in their walls. Conclusion: Sjögren syndrome accompanying pulmonary amyloidosis and lymphoproliferative disease manifests as multiple, large, thin-walled cysts; multiple nodules; parenchymal opacity; and bronchiectasis. These findings are caused by the interstitial or peribronchial infiltration of mixed amyloid and lymphoproliferative cells.