[Progressive pulmonary fibrosis in a case of Sjögren's syndrome with asbestos exposure].

A 62-year-old man had an occupational history of exposure to asbestos for 27 years (1947-1974). Dry mouth, dry eyes and swollen gums were noted in 1974. Sjögren's syndrome was diagnosed in 1975. The chest radiograph in 1984 showed bilateral pleural thickening and small reticular opacities in the left lower lung field. In 1990, he complained of dyspnea and cough, and diffuse interstitial pneumonia was diagnosed. While being treated for diffuse interstitial pneumonia, pulmonary fibrosis progressed rapidly, and advanced pulmonary fibrosis was obvious in 1992. Laboratory data showed hyper gamma-globulinemia (36.8%) and a high level of IgG (3772 mg/dl) in 1976. These values decreased to within the normal ranges during the subsequent clinical course. The results of lymphocyte subset counts in 1988 were normal. With the progression of diffuse interstitial pneumonia in 1990, the lymphocyte subset counts showed a low percentage of CD4 (19.2%), a low CD4/8 ratio (0.46), and a low percentage of CD20 (26.8%). In 1992, as pulmonary fibrosis progressed despite treatment, the disorder of lymphocyte subsets became worse. The CD4 percentage was very low (5.0%), as was the CD4/8 ratio (0.08), and the percentage of CD20 (1.4%); the CD8 percentage was high (64.7%), as was the percentage of Leu 7 (49.0%). These immunologic and pulmonary changes could be not explained by Sjögren's syndrome. Determining what factor induced these changes is difficult, but asbestos exposure is a likely cause.