Yoko Muramatsu

Effectiveness of combined therapy with pirfenidone and inhaled N-acetylcysteine for advanced idiopathic pulmonary fibrosis: a case-control study.

Treatment with pirfenidone may slow the decline in vital capacity and increase progression‐free survival (PFS) in idiopathic pulmonary fibrosis (IPF). The effects of combination therapy with inhaled N‐acetylcysteine (NAC) and pirfenidone are unclear. We assessed the effects of this combination therapy in patients with advanced IPF.

Antioxidant nutrients in plasma of Japanese patients with chronic obstructive pulmonary disease (COPD), asthma-COPD overlap syndrome, and bronchial asthma.

Few studies to date have investigated the antioxidant nutrients such as vitamin C (ascorbic acid), vitamin E (α‐tocopherol), retinol and carotenoids in plasma from patients with pulmonary disease in Japan. To clarify the role of antioxidant nutrients such as vitamin C, vitamin E, retinol and various carotenoids in plasma of Japanese patients with chronic obstructive lung diseases (COPD), asthma‐COPD overlap syndrome (ACOS) and/or bronchial asthma (BA), we compared to healthy elderly controls.

Malignant pleural mesothelioma mimicking the intrapulmonary growth pattern of epithelioid hemangioendothelioma

To the Editor: Malignant pleural mesothelioma (MPM) is a rare tumor with a very poor prognosis: median survival time from diagnosis is 9–12 months. The main pathogenic factor is asbestos exposure. In Japan, MPM incidence has been increasing rapidly since the 1960s and is expected to continue rising. Histologically, MPM can be subclassified as epithelioid (60% of cases), biphasic (30%), and sarcomatoid (10%). Some features of MPM can be visualized by computed tomography (CT); most commonly pleural effusion, pleural thickening, volume contraction, involvement of mediastinal pleura, and interlobar fissure. However, MPM is difficult to differentiate from lung adenocarcinoma with pleural dissemination on the basis of CT findings alone. MPM typically manifests locally in the hemithorax and spreads to adjacent lung tissues, predominantly by direct invasion. Parenchymal involvement in the form of pleural-based masses extending into the lung was reported in 6–10% of MPM cases in which unusual patterns of parenchymal growth were found. The unusual parenchymal growth patterns were classified as epithelioid hemangioendothelioma (EHE)-like, bronchioloalveolar carcinoma-like, desquamative interstitial pneumonia-like patterns. This EHE-like pattern was similar to an intra-alveolar growth pattern. Only sarcomatoid MPM has been reported to mimic EHE. A 72-year-old Japanese man was hospitalized for progressive dyspnea on exertion, and a chest X-ray showed right pleural effusion. He was a heavy smoker (smoking index: 1500) and had been exposed to asbestos for over 30 years at his job. Physical examination of the chest revealed marked reduction of breath sounds on the right side. Chest CT after drainage showed focal pleural thickening near the vertebral body and a peripheral intrapulmonary nodular lesion (diameter, 15 mm) in the posterior basal segment (S10) of the right lung (Fig. 1). In a sample of the pleural effusion, the Cyfra 21-1, cytokeratin 19 fragment (CYFRA) level was 6.7 ng/mL, and the hyaluronic acid level was 28 200 μg/L. Cytologic and bacteriologic examinations of the pleural effusion revealed no evidence of malignancy. Video-assisted thoracoscopic surgery (VATS) was performed to establish a definitive diagnosis. Thoracoscopy showed diffuse fibrous adhesion, multiple small nodular lesions on the parietal pleura, and a solid subpleural nodule in S10 of the right lung, with whitish thickened pleura around the nodule. A pleural biopsy obtained by VATS included several small nodular lesions. A lung biopsy of the nodule in S10 of the right lung was also obtained. Histologic examination of the biopsied parietal pleura showed features typical of biphasic MPM, namely interlacing fascicles of papillotubular formations of epithelioid cells and storiform formation of spindle cells in one lesion. Immunohistochemical staining for mesothelial markers was strongly positive for calretinin and weakly positive for Wilms tumor 1 (WT-1) and D2-40 in the epithelioid area. In the sarcomatoid area, staining was positive for calretinin, WT-1, and D2-40. Both areas were negative for carcinoembryonic antigen (CEA) and thyroid transcription factor-1 (TTF-1). The intrapulmonary nodule in the right posterior basal segment was biphasic, and both areas contained spindle and epithelioid cells. The tumor spread diffusely over the visceral pleura and infiltrated the peripheral lung. A few intracytoplasmic vacuoles were present in tumor cells in the epithelioid area. Elastica Van Gieson (EVG) staining revealed that the nodule comprised centrally hyalinized stroma and encircling