[A rare case of primary Sjögren's syndrome complicated by secondary lymphoid interstitial pneumonia]

Yasutaka Watanabe, Masato Moriguchi, Sayaka Otani, Chihiro Miwa, Hiroyoshi Tsubochi, Shunsuke Endo, Mitsuhiro Nokubi, Yoshinori Kawabata, Shinichiro Koyama
Nihon Kokyūki Gakkai Zasshi, the Journal of the Japanese Respiratory Society 2009, 47 (7): 597-601
A 61-year-old woman was referred to our hospital because of an abnormal chest X-ray shadow. She was diagnosed as having primary Sjögren's syndrome based on sicca syndrome, polyclonal hyper-gamma-globulinemia, and positive results for anti-Ro/SS-A and anti-La/SS-B antibodies. Her chest CT scan revealed bilateral ground-glass shadows and septal thickning in the lower lung field (Fig. 2a). Biopsy specimens obtained by video-assisted thoracoscopy (VATS) showed a dense interstitial lymphoid infiltrate, including lymphocytes and plasma cells, predominantly in the alveolar septae (Fig. 3, a-c). Malignant lymphoma was excluded on the basis of immunohistopathological studies (Fig. 4). A diagnosis of lymphoid interstitial pneumonia was made according to the 2002 American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification (International Classification). After administration of prednisolone and azathioprin, the ground-glass shadows on the chest CT rapidly improved (Fig. 2b). The clinico-radiologic-pathologic diagnosis based on the VATS procedure and the International Classification is appropriate and effective for treating collagen vascular disease-associated interstitial lung disease.

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