[Primary Sjögren's syndrome with lymphocytic interstitial pneumonia, autoimmune hemolytic anemia and an endobronchial amyloid nodule]

Junya Kitada, Gen Yamad, Takashi Ochi, Shintaro Tanaka, Takayuki Itoh, Atsushi Watanabe, Masaaki Satoh, Hiroki Takahashi
Nihon Kokyūki Gakkai Zasshi, the Journal of the Japanese Respiratory Society 2008, 46 (11): 960-5
A 73-year-old woman was admitted to our hospital for examination of anemia, dry feeling of oral and eyes with chest abnormal shadow in October, 1999. Chest radiograph showed interstitial shadows difference and CT showed small nodular opacities in both lung fields. Laboratory examination showed direct Coombs test was positive, and the serum levels of indirect bilirubin and haptoglobin were elevated. Anti-SS-A antibody and anti-SS-B antibody were positive with hypergammaglobulinemia. The serum levels of SP-D and KL-6 were elevated. Lip biopsy showed atrophy and lymphocyte infiltration of the salivary glands. According to these findings, she was diagnosed as primary Sjögren's syndrome with autoimmune hemolytic anemia. In addition, bronchoscopic examination showed an endobronchial polyp in the right B8, and the biopsy specimen showed AL type amyloid deposits. In order to obtain pathological diagnosis of lung lesions, we performed lung biopsy by video-assisted thoracoscopic surgery. The biopsy specimen showed severe infiltration of lymphocytes and the plasma cells around peribronchiole with lymphoepithelial lesions, suggesting malignant lymphoma. However, immunohistochemistry did not show monoclonal profile and Southern blot hybridization assay demonstrated no rearrangement of JH gene. Lung lesions were diagnosed as lymphocytic interstitial pneumonia due to Sjögren's syndrome. Steroid therapy was performed and followed by improvement of anemia and pulmonary lesions. Malignant lymphoma has not been involved for eight years after a diagnosis.

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