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 Sjogren syndrome accompanying pulmonary amyloidosis and lymphoproliferative disease and to compare these with histopathologic findings.


METHODS
The thin-section CT findings of 5 women (age range: 42-59 years, mean age=50 years) with primary Sjogren 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.


CONCLUSIONS
Sjogren 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.
Jeong
Yeon Joo
YJ
Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Lee
Kyung Soo
KS
Chung
Man Pyo
MP
Han
Joungho
J
Chung
Myung Jin
MJ
Kim
Kun-Il
KI
Seo
Joon Beom
JB
Franquet
Tomas
T
D000328:Adult; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000473:pathology; D001706:Biopsy; D001980:Bronchi / Q000473:pathology; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000473:pathology; D001995:Bronchography; D002114:Calcinosis / Q000000981:diagnostic imaging / Q000473:pathology; D003560:Cysts / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods; D008171:Lung Diseases / Q000000981:diagnostic imaging* / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000000981:diagnostic imaging* / Q000473:pathology; D008875:Middle Aged; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000000981:diagnostic imaging* / Q000473:pathology; D020775:Thoracic Surgery, Video-Assisted; D014057:Tomography, X-Ray Computed / Q000379:methods*