[Primary Sjögren's syndrome with lymphocytic interstitial pneumonia and pulmonary multiple cystic lesions].

We report a case of primary Sjögren's syndrome with lymphocytic interstitial pneumonia and multiple cystic lesions. The patient was a 64-year-old woman. Abnormal chest shadows were detected by x-ray and computed tomographic (CT) examinations. The patient had no family history of disease and had never smoked. She had complained of dryness in the eyes and mouth for about 10 years. Laboratory tests were positive for anti-nuclear antigen, anti-SS-A antigen, and anti-SS-B antigen. Sialography revealed marked destruction of the salivary glands, yielding a diagnosis of Sjögren's syndrome. Chest X-ray films and CT scans showed multiple cystic lesions in both lungs, measuring from a few mm to 3 cm in diameter, as well as fine centrilobular nodules. Slight anemia and hyper gamma globlinemia were also detected. Pulmonary function tests showed mild obstructive disturbance. Bronchoalveolar lavage analysis disclosed an elevated lymphocytic fraction (28.6%), but transbronchial lung biopsy provided no adequate specimens for diagnosis. Thoracoscopic lung biopsy specimens demonstrated marked infiltration of lymphocytes and histiocytes through the interstitium of alveolar walls and peri-bronchovascular sheath, with some lymphoid follicles. The overall appearance was compatible with lymphocytic interstitial pneumonia. The cysts themselves were nonspecific, and no cellular infiltration was noted in the cyst walls. Because of the predominantly peribronchial distribution of the lesions, we suspected that the cysts were formed by the check valve mechanism. However, no definitive evidence was obtained.