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Fibrosing mediastinitis (FM) is a rare fibroinflammatory disease of the mediastinum with an etiology and clinical features that vary by world region. The characteristics of FM in Japan are still unknown. We herein report two Japanese patients with FM who were treated with corticosteroids and responded well. We also reviewed the Japanese literature on PubMedⓇ and summarized the characteristics of 27 Japanese FM patients, including our two patients. In Japan, the predominant cases were those without a specific cause, were diffusely distributed, and responded well to corticosteroid therapy.

A 70-year-old woman was admitted to our hospital for dyspnea and a fever of 2 weeks duration. Chest imaging showed bilateral infiltration, and a rapid diagnostic test for influenza virus, Mycoplasma pneumoniae, Streptococcus pneumoniae, and Legionella spp. was negative. She was intubated and mechanically ventilated and underwent bronchoalveolar lavage. Bronchoalveolar lavage fluid yielded no significant pathogens, and the multiplex polymerase chain reaction test was positive only for human bocavirus. Specific antibodies against significant pathogens were not increased in paired sera, so we diagnosed her with primary human bocavirus pneumonia.

Objective The clinical characteristics and chest imaging findings of viral pneumonia and several interstitial lung diseases (ILDs) overlap, and viral pneumonia may be underrecognized and misdiagnosed as certain ILDs. To clarify the frequency of viral pneumonia among patients with acute progressive clinical courses that required a differential diagnosis between ILDs and pneumonia, and to determine the most frequent ILDs misdiagnosed in cases of viral pneumonia. Patients and Methods We retrospectively analyzed patients hospitalized from 2010 to 2017 with an acute clinical course (≤30 days) who underwent bronchoalveolar lavage (BAL) for the differential diagnosis of infection and ILDs. We performed a multiplex PCR for respiratory viruses using the patients' preserved BAL fluid. The final diagnosis was made by a multidisciplinary approach and after considering the PCR results. The diagnosis at discharge was compared to the final diagnosis. Results Among the 109 patients, 53 were diagnosed with viral pneumonia. Viral pneumonia and other diseases showed some differences in symptoms and laboratory data; however, the differences were small or overlapped. Viral pneumonia was misdiagnosed on discharge as acute fibrinous organizing pneumonia, cryptogenic organizing pneumonia, or chronic eosinophilic pneumonia (AFOP/COP/CEP) (n=22), acute interstitial pneumonia (n=5), connective tissue disease-related ILDs (n=3), unclassifiable interstitial pneumonia (n=2), drug-induced ILD (n=1), and pneumonia (n=20). Conclusion Approximately half of the patients who underwent BAL had viral pneumonia. The most common ILD-related misdiagnoses were AFOP/COP/CEP. Differences in symptoms and laboratory findings between viral pneumonia and other diseases were small, and viral pneumonia should be included in the differential diagnosis when physicians encounter cases in which the abovementioned ILDs are suspected.

A 63-year-old woman presented to our hospital for cough, sputum, and abnormal shadows on chest X-ray. Schizophyllum commune was isolated from mucous plugs. Positive specific IgE and IgG against the fungi, elevated serum IgE, and mucous plugs with typical histologic findings of allergic bronchopulmonary mycosis (ABPM) led to the diagnosis of ABPM due to S. commune. We initially administered itraconazole unsuccessfully. Changing the antifungal agent to voriconazole resulted in improvement of the symptoms and chest imaging findings. Her ABPM has not relapsed for two years since the cessation of voriconazole, which was administered for one year.

[Objective] Presently, the predominant subtypes of influenza viruses in the world, except for those in local epidemics, include influenza pandemic H1N1 2009 (pH1N1), H3N2, and B viruses. There are few reports on the differences in the clinical features, radiographic findings, treatment, and outcomes of influenza virus-associated pneumonia among these three viral subtypes. The purpose of this study was to investigate whether the clinical features, radiographic findings, treatment, and outcomes differ among the viral subtypes. [Methods] We retrospectively analyzed 96 patients with influenza virus-associated pneumonia whose viral subtypes were clarified. [Results] Patients with pH1N1 virus-associated pneumonia tended to be young. The frequency of primary viral pneumonia differed among the virus-associated pneumonia subtypes (pH1N1, 80%; H3N2, 26.5%; and B, 31%). Patients with pH1N1 virus-associated pneumonia more frequently showed bilateral ground-glass opacities (GGOs), which affected more lobes than in patients with H3N2 and B virus-associated pneumonia. However, patients with H3N2 virus-associated pneumonia showed a higher frequency of consolidation and diffuse bronchial wall thickening than did the patients with pH1N1 virus-associated pneumonia. The severity and mortality did not differ among the three pneumonia subtypes. [Conclusion] In the patients who developed influenza virus-associated pneumonia, those with pH1N1 virus-associated pneumonia frequently developed primary viral pneumonia resulting in bilateral and broad areas of GGOs on imaging, whereas patients with H3N2 virus-associated pneumonia frequently showed consolidation and diffuse bronchial wall thickening on pulmonary imaging.

A 58-year-old man presented with right chest pain, anorexia, general malaise, and fever. Chest computed tomography showed a mass lesion with right middle lobe atelectasis. The bronchoscopy showed polypoid lesions with a smooth surface in each right middle lobe bronchial lumen. The histopathology revealed the dense accumulation of histiocyte-like cells with foamy cytoplasm under the bronchial epithelium along with yeast-like fungi stained positively with both Alcian blue and Grocott's stains. Cryptococcus neoformans was cultured from the bronchial washings. We diagnosed the patient with pulmonary cryptococcosis with endobronchial lesions. The fluconazole treatment was changed to liposomal amphotericin-B and flucytosine after the diagnosis of cryptococcal meningitis. The minimum inhibitory concentration of the fungi suggested resistance to fluconazole and flucytosine. The lesion regressed after these treatments.

A 76-year-old woman suffering from granulomatosis with polyangitis (GPA) developed organizing pneumonia with positive antineutrophil cytoplasmic antibodies and microscopic hematuria. Prednisolone improved the hematuria and radiological findings; however, after tapering the dose of prednisolone, a posterior left atrial wall mass was detected in association with a fever. Both regressed spontaneously, although secretory otitis media and sinusitis were noted; the resected sinusitis specimen exhibited vasculitis highly suggestive of GPA. The clinical picture of GPA with multi-organ involvement can vary. Recognizing the various manifestations of GPA is therefore necessary in order to provide an appropriate diagnosis and disease management.

A 73-year-old woman with rheumatoid arthritis treated with methotrexate and prednisolone was admitted with dyspnea and ground-glass opacity on chest CT. We diagnosed her with Pneumocystis jirovecii pneumonia (PCP) based on a positive PCR analysis of Pneumocystis jirovecii and the presence of cysts in bronchoalveolar lavage fluid. The PaO2 was 74.7 Torr on room air, and treatment with sulfamethoxazole-trimethoprim only was initiated. The hypoxemia and ground-glass opacity increased on hospital day 3, and the administration of adjunctive steroid therapy resulted in an improvement in the patient's condition. Although patients with PCP with HIV infection and hypoxemia are often treated with adjunctive steroid therapy to prevent adverse immune reactions, the efficacy of additive steroid administration in case of non-HIV PCP has not been established.

Presently, 6 cases of IgG4-related pleuritis have been reported. We encountered a patient who developed chylothorax due to IgG4-related disease. To our knowledge, such patients have not been reported. This patient developed right-sided chylothorax and left-sided non-chylothorax lymphocyte-predominant pleuritis. Elevated serum and pleural IgG4 concentrations and histopathological analysis of pleural biopsy confirmed the diagnosis of IgG4-related pleuritis. Left-sided pleuritis improved with corticosteroid therapy, but right-sided chylothorax persists. IgG4-related disease can be one cause of chylothorax.

The leading subtype of lung cancer, adenocarcinoma, received a new classification in 2011 when multifocal, pneumonic-type mucinous tumors, formerly called mucinous bronchioloalveolar carcinomas, were reclassified as "invasive mucinous adenocarcinomas." These tumors appear to be less sensitive to chemotherapy than other non-small cell lung cancers. We herein report two cases of invasive mucinous adenocarcinoma that showed a dramatic radiologic response to combination therapy with cisplatin, pemetrexed and bevacizumab. Recent data suggest that the use of paclitaxel-based chemotherapy is an acceptable therapeutic strategy in cases of invasive mucinous adenocarcinoma. The cases reported here and preclinical findings suggest the therapeutic efficacy of cisplatin, pemetrexed and bevacizumab in treating such tumors.

A 68-year-old woman without asthma presented with a cough and abnormal shadows on a chest X-ray. Computed tomography showed right middle lobe atelectasis and centrilobular nodules with a tree-in-bud appearance in the other lobes. The patient's sputum repeatedly yielded positive cultures of nontuberculous mycobacteria (NTM); however, no fungi were detected. A transbronchial biopsy showed allergic mucin with eosinophils, although the findings were not diagnostic. We suspected that the patient had pulmonary mycobacteriosis; however, treatment for this condition failed. We then performed thoracoscopy for further evaluation and treatment of the NTM infection. The resected specimen obtained from the right middle lobe exhibited the characteristic findings of allergic bronchopulmonary mycosis without evidence of mycobacterial infection. The administration of corticosteroids and itraconazole resulted in improvement of the patient's condition. The NTM appeared to be simply a coincidental colonization of the resected middle lobe bronchus. The absence of asthma, the inability to isolate fungi and the repeated isolation of NTM made it difficult to differentiate allergic bronchopulmonary aspergillosis from NTM infection.

A 38-year-old man with Marfan syndrome underwent an aortic replacement with an artificial aortic valve at 27 years of age and an aortic graft at 31 years of age. In 2011, he was diagnosed as having chronic necrotizing pulmonary aspergillosis (CNPA). He developed a fever and an increased sputum volume and was admitted to the hospital in 2012. Contrast-enhanced CT showed an irregularly shaped nonenhanced structure in the aortic graft. He died on hospital day 31. From the autopsy findings, we speculated that an infiltration by the CNPA lesion into a pulmonary vein was followed by the hematogenous formation of a fungus ball in the aortic graft.