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type:Case Report, Recently, immunoglobulin (Ig) G4-related diseases such as autoimmune pancreatitis (AIP), sclerosingsialadenitis, retroperitoneal fibrosis, and sclerosing cholangitis have been reported. IgG4-related diseases arecharacterized by high serum IgG4 concentrations, sclerosing inflammation with numerous IgG4-positiveplasma cells, and steroid sensitivity, irrespective of their organ of origin. We describe a case of inflammatorylung disease and retroperitoneal fibrosis, suggested to involve IgG4. The patient was a 76-year-old man. Acomputed tomographic scan of the chest showed nodular air-space consolidation in the left upper lobe. Theserum IgG4 concentration was abnormally elevated, but there was no evidence of AIP. Bilateral hydronephrosisassociated with thickened soft tissue around the abdominal aorta had been diagnosed previously. Hehad undergone surgery, and retroperitoneal fibrosis was diagnosed histologically (hematoxylin and eosinstain). Histological examination of bronchoscopic specimens taken from the left S3 region showed mononuclear-cell infiltration of the fibrotic bronchial wall, including many IgG4-positive plasma cells. Specimens ofthe region affected by retroperitoneal fibrosis were retrospectively reanalyzed, and the cells were positivefor IgG4 on immunostaining, similar to the lung tissue. The patient responded to treatment with corticosteroids.In conclusion, the present case shared many clinical and clinicopathological similarities with systemicIgG4-related autoimmune disease. To our knowledge, however, this is the first reported case of inflammatorylung disease with retroperitoneal fibrosis in a patient with systemic IgG4-related autoimmune disease., identifier:6, identifier:KJ00006372599

type:Original, Background and objective:The calcium-binding protein S100A4 belongs to the S100 family and is involved in fibrotic and inflammatory processes, in which tissue remodeling, cell motility, and epithelialmesenchymal transition play major roles. Cytoplasmic S100A4 is a marker of lung fibroblasts in pulmonary fibrosis;however, the effects of exogenous S100A4 on fibrotic and inflammatory processes in pulmonary fibrosis are unclear. This study examined the effects of exogenous S100A4 protein in mice with bleomycin-induced pulmonary fibrosis.Methods:Bleomycin was administered to mice by intratracheal instillation on day 1. Intratracheal S100A4 protein was administered 4 times after bleomycin treatment. Bronchoalveolar lavage fluid was obtained and lung histological examinations were performed on day 14 after bleomycin administration. Lung tissue was homogenized on the same day to assess the mRNA expression of cytokines, fibroblast growth factors, and S100A4.Results:Unexpectedly, we observed that the administration of exogenous S100A4 protein apparently reduced lung fibrosis in bleomycin-treated mice. In addition, the levels of lymphocyte accumulation and insulin-like growth factor-1 mRNA were significantly reduced in bleomycin-treated lung by S100A4 administration.Conclusions:Exogenous S100A4 protein attenuates bleomycin-induced pulmonary fibrosis in mice by reducing lymphocyte function and the levels of fibroblast growth factors., identifier:2, identifier:KJ00010247939

type:Case Report, Benign metastasizing leiomyoma (BML) is a very rare disease, and although it was reported as early as1939 to result from metastasis of benign uterine myoma to the lungs and lymph nodes, its pathology remainsobscure. Here, we describe a case of pulmonary BML occurring after uterine myomectomy in a42-year-old woman. She presented with a 2-week history of dry cough on exertion. Chest radiography andcomputed tomography( CT) revealed bilateral multiple nodular lesions. The patient had a history of uterinemyoma and previously underwent myomectomy. For definitive diagnosis, lung biopsy was performed byvideo associated thoracoscopic surgery. Histopathologic examination of biopsy specimens revealed pulmonaryBML occurring after uterine myomectomy. For treatment of the pulmonary BML, gonadotropin-releasinghormone was initially administered, and 1 month later the patient underwent complete hysterectomyand bilateral salpingo-oophorectomy. Chest CT 6 months after surgery showed that the size and number oflung multiple nodular lesions did not increase compared with those before surgery. In future studies, we aimto investigate a larger number of pulmonary BML cases, as well as establish specific treatments and investigatethe prognosis of the disease.Abbreviations:BML:benign metastasizing leiomyomaSS:Sjögren's syndromeCT:computed tomographyH&E:hematoxylin and eosinVATS:video associated thoracoscopic surgeryCA:carbohydrate antigenIg:immunoguloblina SMA:a smooth muscle actin, identifier:12, identifier:KJ00006378199