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A 66-year-old man was found to have a very small ground-glass opacity in the apex of the left lung. Because the ground-glass opacity had slightly enlarged after 2 years, video-assisted thoracic surgery (VATS) biopsy was performed. The histological findings showed the alveolar spaces to be filled with PAS-positive granular materials, so pulmonary alveolar proteinosis was diagnosed. Although his bronchoalveolar lavage fluid (BALF) did not have a milky appearance, his serum and BALF GM-CSF autoantibody and serum KL-6 levels were elevated. Asymptomatic pulmonary alveolar proteinosis may appear as very small ground-grass opacities.

We evaluated the consistency of the A-DROP system for community-acquired pneumonia (CAP) and its outcomes, and developed a new severity classification of community-acquired pneumonia using nonlinear discriminant analysis. A total of 615 patients with CAP were enrolled between 2004 and 2009. A poor outcome was defined as patients requiring ventilation and/or death from CAP. We investigated the influence of prognostic factors on CAP severity and outcome using a logistic regression model to obtain the coefficient, and a contingency table. The optimal cutoff points for age and BUN were calculated from receiver-operating characteristic (ROC) curves. The influence of respiratory failure was approximately twice that of other prognostic factors. The optimal cutoff point for age was 83 years old, and that for BUN was 23mg/dl. We found inconsistencies in the equivalence of all prognostic factors and the addition-scoring method in predicting outcome. To ensure consistency between the A-DROP system and outcome, we believe that the weight of respiratory failure, threshold of classification, and cutoff points for age and BUN should be revised.

A 56-year-old woman who had suffered from systemic lupus erythematosus and Sjögren syndrome was admitted complaining of persistent cough. Chest X-ray films showed an infiltrative shadow in the right middle lung field. Her serum PR3-ANCA titer was high, and granulomatous inflammation with Langhans giant cell was noted in a transbronchial biopsy specimen. About 3 months later, purulent sputum and high grade fever developed, with a new infiltrative shadow in the left upper lung field noted on a chest X-ray film. We treated her based on a diagnosis of bacterial pneumonia caused by methicillin-resistant Staphylococcus aureus, but her condition did not improve. We finally gave her a diagnosis of pulmonary-limited Wegener's granulomatosis. Her condition improved with the administration of sulfamethoxazole-trimethoprim, prednisolone and cyclophosphamide. We report a case of pulmonary-limited Wegener granulomatosis which mimicked bacterial pneumonia caused by methicillin-resistant Staphylococcus aureus. This case suggests that Wegener's granulomatosis should be considered on encountering pneumonia caused by Staphylococcus aureus.

A 56-year-old man with chief complaints of dry cough and dyspnea was admitted. He had severe hypoxemia, and his chest radiographs showed enhancement of pulmonary artery opacities with multiple defects on pulmonary blood flow scintigraphy. Enhanced computed tomography (CT) revealed swelling of the mediastinum and hilar lymph nodes, but no apparent thrombi in the pulmonary arteries was seen. A biopsy specimen of a left neck lymph node showed poorly differentiated adenocarcinoma, including signet-ring cell carcinoma components, but the origin was unclear. Despite receiving chemotherapy, his respiratory condition worsened, and he died 3 days after admission. Routine autopsy failed to clarify the tumor origin, but a detailed dissection of specimens confirmed early gastric cancer. Additionally, pathology of the pulmonary arteries was compatible with pulmonary tumor thrombotic microangiopathy (PTTM). PTTM is a rare condition characterized by the presence of diffuse thrombotic microthrombi and fibrocellular intimal proliferation in the pulmonary vasculature. Accompanied with early gastric cancer, this is an extremely rare but important case of PTTM.

A 48-year-old man, who had worked as a welder for 30 years, was admitted to our hospital with bloody sputum. His chest CT scan showed diffuse centrilobular micronodules and fungus balls within cavities in the apices of both lungs. We diagnosed pulmonary aspergilloma associated with welder's lung in the apices of both lungs, by bronchoscopy. He was treated with oral antifungal drugs for 1 month, but he had massive hemoptysis. Bronchoscopy showed that the hemoptysis originated from the right upper lobe. We performed right upper lobectomy after right bronchial and intercostal arterial embolization. About 2 months after surgery, he had bloody sputum again. We then performed left bronchial and intercostal arterial embolization because bronchoscopy showed that the bloody sputum originated from the left upper lobe. His bloody sputum disappeared after the last embolization treatment. We report a rare case of pulmonary aspergilloma associated with welder's lung, with a discussion based on a review of the literature.

A 73-year-old man was admitted with high fever and right chest pain. Chest X-ray showed a rapidly growing mass shadow in the right lower lung field. The patient had been in remission for malignant lymphoma and had developed interstitial pneumonia and diabetes mellitus following 1 year of corticosteroid therapy. His illness was diagnosed as invasive aspergillosis because of a high level of beta-D-glucan and cultured Aspergillus fumigatus in the sputum. He was treated with a combination of micafungin and itraconazole. However, because these agents did not improve his clinical condition, transbronchial lung biopsy was performed. Histologically, Mucor hyphae were detected in these specimens. Micafungin and itraconazole were stopped and infusion of liposomal amphotericin B was initiated. Because his condition worsened, a right lower lobectomy was performed. Rhizopus Oryzae was detected in the lung tissue. We report a case of pulmonary mucormycosis in which mixed infection with A. fumigatus was suspected. Pulmonary mucormycosis is a life-threatening infection in which it is rare that an antemortem diagnosis is established and organisms are isolated. We believe diagnostic tests should be performed aggressively, even when pulmonary aspergillosis is suspected.

An 83-year-old woman was referred to our hospital with dyspnea on exertion and right pleural effusion. At the age of 69, she had been given a clinical diagnosis of sarcoidosis due to uveitis, bilateral hilar lymphadenopathy, bilateral multiple nodular shadows on chest images, and serum angiotensin-converting enzyme (SACE) level elevation. Remission was spontaneous. The pleural effusion was exudative lymphocyte-rich. On thoracoscopy, the macroscopic appearance of the parietal pleura was telangiectasia without nodular lesions and the pleural biopsy specimens revealed non-caseating epitheloid cell granulomas whose cultures were negative for acid-fast bacilli and fungi. A tuberculin skin test and QFT-2G were negative, thus we diagnosed sarcoidsis pleurisy.

The definitive diagnosis of idiopathic pulmonary fibrosis (IPF) requires a pathologic pattern of usual interstitial pneumonia (UIP). However, it is not clear that whether there is a difference in outcome between patients with IPF diagnosed by clinical findings, and those diagnosed by pathological findings. We investigated differences in survival between patients diagnosed with IPF based on clinical findings, and patients who underwent surgical biopsy for a pathological diagnosis of IPF/UIP based on findings showing a UIP pattern. Sixty-one patients who received a clinical diagnosis of IPF were selected by random sampling. Fifty-nine patients underwent open lung biopsy or video assisted thoracoscopic biopsy and receive a pathological diagnosis of IPF/UIP. At diagnosis, 23 clinical features were identified and the hazard ratio of pathological diagnosis was estimated using the Cox proportional hazard model adjusted for those features that differed between the two groups. The adjusted hazard ratio of pathological diagnosis, taking these factors into account, was 1.09 (95% confidence interval, 0.42 to 2.80, p = 0.861) to about 1.0. Therefore, it is reasonable to diagnose IPF based on clinical findings from the perspective of outcome.

A 49-year-old-man was admitted to our hospital because of cough, wheezing, and dyspnea. Chest CT showed bilateral bronchocentric nodules and increased bronchial thickness. And he was given a diagnosis of bronchial asthma. However, another small airway disease, such as diffuse panbronchiolitis (DPB) was also suspected, and surgical lung biopsy was performed. The biopsy specimens showed a prominent peribronchial lymphoplasmacytic and eosinophilic infiltration with IgG4-positive plasma cells. IgG4-positive plasma cells may play a role in the pathogenesis of bronchiolar diseases associated with allergic airway inflammation.

A 70-year-old man was admitted to our hospital because of multiple ground-glass opacities on his chest CT scan. A transbronchial lung biopsy specimen revealed signet-ring cell carcinoma infiltrating into the alveolar septa. The histological findings of the carcinoma obtained from the lung were very similar to those of his gastric carcinoma which had been resected at age 66. Immunohistochemical staining of the cancer cells were positive for keratin 7 and keratin 20, therefore a metastatic lung tumor from gastric cancer was diagnosed. Although multiple, well-defined nodules are typically considered to be the presentation of pulmonary metastases, clinicians should also be aware that multiple, ill-defined ground-glass opacities can also be recognized as pulmonary metastasis.

We report 3 cases of occupational hypersensitivity pneumonitis in citrus farmers. They were admitted to our hospital with abnormal chest shadows with coughs, fevers and breathlessness between January and February, but their symptoms disappeared with isolation from their workplace. The diagnoses were comprehensively confirmed by their occupational histories, radiological findings, and the positive findings of environmental provocation tests. Although we cannot clearly determine the pathogenic antigen of this hypersensitivity pneumonitis, Aspergillus and Penicillium might be the causative agents, because they were detected in the patients' workplaces and in double immunodiffusion tests. In Ouchterlony's immunodiffusion test, these antigens were positive in some patients.

Interstitial pneumonia in a 54-year-old woman was diagnosed in April 2007, without symptoms. She was admitted to our hospital in February 2009 because chest radiography showed gradual worsening. On physical examination, she had fine crackles on chest auscultation and "mechanic's hands", without arthritis, myopathy, or Reynaud disease. On laboratory tests, antinuclear antibody (speckled and cytoplasmic pattern) and anti SS-A antibody were positive. Chest CT showed ground-glass opacities in both lower lobes with volume loss and she had a restrictive disorder on a pulmonary function test. Bronchoalveolar lavage was within almost normal limits. For a definitive diagnosis, video associated thracoscopic surgery was performed. The histopathological findings showed a fibrotic nonspecific interstitial (f-NSIP) pattern. Corticosteroid therapy (prednisolone, initially 40 mg/day) was started, and the chest radiography improved. Later, an RNA immunoprecipitation test was positive for anti-signal recognition particle (SRP) antibody. This is a rare case of interstitial pneumonia with anti-SRP antibody without myopathy.

A 19-year-old woman with anorexia nervosa (body weight 25 kg) was admitted to our hospital, showing a cavitary shadow in the left upper lung field on a chest radiograph film. We diagnosed a pulmonary abscess caused by Pseudomonas aeruginosa. A few days later, exacerbation, including enlargement of the cavitary shadow, ipsilateral pleural effusion and bilateral infiltrations, were observed. Finally, by using antibiotics such as Doripenem (DRPM) and Ciprofloxacin (CPFX), we were able to treat the disease by bronchoscopic and thoracic drainage. This case suggests we should take carefully treat anorexia nervosa patients with pulmonary infection.

A 59-year-old woman began to complain of cough, dyspnea, and fever 2 weeks after 3 courses of chemotherapy with cyclophosphamide (800 mg) and adriamycin (80 mg) for breast cancer. Chest radiography showed diffuse ground-glass shadows in the central areas of bilateral lung fields. Chest CT also showed diffuse ground-glass opacities in the central areas of bilateral lung fields. Arterial blood gas analysis revealed mild hypoxia. A lymphocyte stimulation test with peripheral blood lymphocytes for cyclophosphamide was negative. A mild increase of lymphocytes was observed in the cell population of bronchoalveolar lavage fluids and no microorganisms were detected. Her respiratory condition improved after glucocorticoid therapy. A rechallenge test with cyclophosphamide was performed after obtaining informed consent. There was no significant change in symptoms or chest radiography results 24 hours after intravenous administration of cyclophosphamide (100 mg). However, a transient decrease of PaO2 with peripheral eosinophilia was observed. Thus, a diagnosis of early-onset cyclophosphamide-induced pneumonitis was established.

A 72-year-old-man was admitted to our hospital because of an abnormal chest shadow. Histological findings from video-assisted thoracoscopic surgical lung biopsy showed desquamative interstitial pneumonia (DIP). Although DIP is classified as the idiopathic interstitial pneumonias (IIPs), it is widely accepted that the cause of DIP might be associated with cigarette smoking. The patient had chronic hepatitis C virus (HCV) infection; however he did not have a history of active or passive smoking. An immunologic response to HCV infection may have a role in the pathogenesis of DIP in patients with chronic HCV. DIP should be included in the differential diagnosis of diffuse parenchymal lung disease in patients with HCV infection.

A 25-year-old man was referred to our hospital because of cough and an abnormal shadow on chest X-ray film showing bilateral hilar lymphadenopathy accompanied by multiple nodules in both lung fields. A transbronchial lung biopsy demonstrated non-caseating epithelioid cell granulomas, and we diagnosed sarcoidosis. He was observed without medication for 18 months, however, his chest X-ray film findings gradually worsened, and bilateral pleural effusion appeared. The pleural effusion consisted of exudative fluid with prominent lymphocytes, and ADA level was elevated to 57.0U/l. Thoracoscopy demonstrated multiple whitish granulations on the parietal and visceral pleura. The pleural biopsy specimens exhibited non-caseating epithelioid cell granulomas, and there was no evidence of acid-fast bacilli. Based on these findings, pleural sarcoidosis was diagnosed. He was treated with 30 mg oral prednisolone daily, and both pleural effusion and nodules of lung fields on chest X-ray film subsided. Sarcoidosis with bilateral pleural effusions is rare, and we discuss this condition in relation to the pertinent literature.

A 67-year-old woman was admitted to our hospital for weakness in her right hand. MRI showed multiple cerebral infarctions and ultrasonic cardiography revealed vegetation on her aortic valve, so embolic stroke was diagnosed. Though she was afebrile and her vital signs were normal, chest CT revealed several enlarged mediastinal lymph nodes and a small nodule in the left lower lobe of the lung. Stage III adenocarcinoma of the lung was diagnosed, and the cause of her cerebral infarctions was found to be nonbacterial thrombotic endocarditis (NBTE). NBTE is known as the cause of embolic stroke among patients with advanced cancer, particularly adenocarcinoma. Prompt initiation of continuous heparin administration is required to interrupt the progress of cerebral thromboembolism in NBTE. In cases of coexisting cancer and embolic stroke, we should consider the possibility of NBTE.

A 52-year old woman admitted to our hospital because of productive cough and dyspnea. Breathing room air arterial blood gases, revealed severe hypoxemia (P/F ratio 185 mmHg) and Chest CT showed diffuse ground glass opacities in both lung fields. Respiratory failure gradually improved after steroid pulse therapy, sivelestat sodium hydrate and antibiotics. However, a mosaic pattern attenuation in the lung parenchyma on Chest CT remained and ultrasonic cardiography showed dilation of right ventricle. Since the presence of V/Q mismatch in pulmonary perfusion and ventilation scintigrams and lupus anticoagulant were detected, we finally diagnosed acute respiratory distress syndrome (ARDS) and pulmonary thromboembolism (PTE) associated with antiphospholipid syndrome (APS). Therefore, this case reminded us that PTE and APS could cause ARDS.

A 54-year-old woman was found to have abnormal shadows on her chest radiograph taken on an annual medical examination. The chest radiograph showed multiple nodules in the bilateral middle and lower lung fields accompanied with bilateral hilar lymphadenopathy. A computed tomography of the neck and chest revealed nodules in her right middle lobe and bilateral lower lobes with an enlarged thyroid. A metastatic malignant disease involving both thyroid and lungs was suspected, therefore thyroid and lung biopsies were performed. The histological examination of the thyroid and the lung specimens revealed non-caseating epithelioid cell granulomas which were compatible with sarcoidosis. Although the thyroid involvement of sarcoidosis is rare, it should be included in the differential diagnosis with patients with thyroid swelling.

A 22-year-old man was admitted to our hospital with fever, cough and dyspnea. His chest radiograph showed diffuse ground-glass attenuation in both lung fields. Arterial blood gas analysis showed hypoxemia (PaO2 28.7 Torr breathing room air) and he required mechanical ventilation within 6 hours after admission. Gomori methenamine silver (GMS) stain of the bronchoalveolar lavage (BAL) fluid smear showed round and indented organisms, and polymerase chain reaction revealed pneumocystis jirovecii in the BAL fluid. The HIV antibody was positive and peripheral blood CD4-positive lymphocytes decreased to 4.0%. Pneumocystis pneumonia complicated with acquired immunodeficiency syndrome (AIDS) was diagnosed. There was no four-fold rise in screen viral titers. We treated him with antibiotics, trimethoprim-sulfamethoxazole, ganciclovir, fos-fluconazole, steroid pulse therapy and sivelestat sodium hydrate. Respiratory failure was relieved within 5 days following treatment. The percentage of neutrophils in the BAL fluid was elevated (44.6%). Neutrophil elastase on admission was increased and improved to the normal range after treatment. Sivelestat sodium hydrate is an anti-neutrophil elastase inhibitor and may be one of the treatment options for acute respiratory failure due to pneumocystis pneumonia in AIDS patients.

A 53-year-old man had received various chemotherapy and steroid treatments for malignant thymoma. He had demonstrated persistent fever since the beginning of January 2006, and chest radiograph showed consolidation in the left middle lung fields. Bacterial pneumonia was suspected, but antibiotics were not effective. He was referred and admitted to our hospital on January 16. Chest radiograph and CT scan on admission showed diffuse ground-glass opacities, consolidation with cavity, and cystic changes. Pneumocystis jiroveci Pneumonia was diagnosed by examination of alveolar lavage. This patient was regarded as immunodeficient because of steroid treatment, low counts of CD4-positive lymphocytes, and the complication of hypogammagloblinemia. We reported this case of a non-HIV patient with atypical images demonstrating Pneumocystis jiroveci pneumonia.

We report a case of pneumonitis induced by Seisin-renshi-in. A 62-year-old man began to complain of cough, dyspnea and fever 45 days after starting to take Seisin-renshi-in for benign prostate hypertrophy. Chest radiograph showed diffuse ground-glass shadows in the bilateral middle and lower lung fields. Chest CT showed diffuse ground-glass opacities in both lung fields. The serum KL-6 level was elevated. Arterial blood gas analysis revealed hypoxia. Lymphocyte stimulation test with peripheral blood lymphocytes for Seisin-renshi-in was positive. A mild increase of lymphocytes, neutrophils and eosinophils was observed in the cell population of bronchoalveolar lavage fluids. Transbronchial lung biopsy specimen showed lymphocytic infiltration into the alveolar septa, desquamative alveolar lining cells and fibrinous exudate in the alveolar spaces. On the basis of a diagnosis of Seisin-renshi-in-induced pneumonitis, steroid therapy was introduced. Three courses of steroid pulse therapy were required because of prolonged hypoxia. His respiratory condition then improved and predonisolone was tapered from 30 mg. We should be aware that over-the-counter drugs could be causal agents of severe pneumonitis.

A 81-year-old woman was admitted to our hospital because of bloody sputum. Chest radiograph showed an enhanced right pulmonary hilum shadow. Computed tomography (CT) revealed localized bronchiectasis of the right lower lung and a nodule protruding into the bronchus. A parenchymal shadow was also seen distal to the nodule. Transbronchial biopsy revealed a cluster of yeast-like fungi and the bronchial lavage culture showed several olive-black colored colonies on Sabouraud agar. Before culture, we empirically administered fluconazole (400 mg/day) on the assumption of candida infection based on the yeast like microscopic findings, however that was not appropriate. Substitution by itraconazole (200 mg/day) made a slight improvement of the shadow on CT. Later polymerase chain reaction of specimens taken by TBLB identified the olive-black fungus as Wangiella dermatitidis. W. dermatitidis is a dematicious fungus sometimes causing tinea nigra or subcutaneous infection. In compromised patients, it causes phaeohyphomycosis especially in the central nerve system. In cystic fibrosis patients, this fungus is recognized to colonize the respiratory tract and rarely causes pneumonia. This case had no previous immunosuppressing disease, except for localized bronchiectasis which was found by CT on admission. We think this is a very rare W. dermatitidis infection case showing a nodular lesion in an immunocompetent patient.

We describe a case of intrapleural rupture of pulmonary arteriovenous fistula and review is such cases previously reported in Japan. A 57-year-old woman was admitted with a sudden onset of right chest pain. Chest radiograph on admission showed right pleural effusion. Thoracentesis revealed hemothorax and subsequently the patient complained of dizziness and went into shock. Chest CT scan revealed a well-defined nodule with a continuous enlarged vessel. Enhanced CT findings suggested a diagnosis of pulmonary arteriovenous fistula projecting into the intrapleural space from the right lower lung. Partial resection of the right lower lung was performed and the histological study confirmed the final diagnosis.

We investigated 23 patients with combined influenza virus and bacterial pneumonia of whom 10 patients were men, 13 were women and the mean age was 78.0 years old. The causative virus of 12 patients was type A, that of 10 patients was type B, and 1 patient was unclassified. S. pneumonia was isolated from 12 patients, S. aureus and H. influenza was isolated from 2 patients. B. Catarrhalis and Milleri spp was isolated from one patient each. The mean interval between onset of influenza and influenza pneumonia was 2.09 days. Fourteen cases developed pneumonia within 2 days after influenza infection. In particular, all nine cases with chronic respiratory disease developed pneumonia within 2 days. All cases except one were treated with oseltamivir and antibiotics, resulting in cure. These data suggest that patients with chronic respiratory disease might develop influenza pneumonia earlier than past reported cases.

A 55-year-old man with a 3-year occupational history of welding was admitted for repeated episodes of fever, cough and dyspnea after inhalation of smoke while welding galvanized steel. A computed tomography (CT) showed diffuse centrilobular nodules, panlobular ground-glass opacity and interlobular septal thickening in both lung fields, and he suffered from hypoxemia (PaO2 = 55.3Torr) while breathing room air. Percentage of lymphocytes in bronchoalveolar lavage fluid increased, and lung biopsy specimens at thoracoscopy revealed lymphocytic alveolitis and organization in air spaces. His symptoms and signs disappeared spontaneously only when he ceased welding. Panlobular ground-glass opacity and interlobular septal thickening improved immediately with oral corticosteroids. Patch tests using metal series gave positive reactions to zinc. We diagnosed this case as hypersensitivity pneumonitis caused by zinc fumes.

A 72-year-old woman with a dry cough and dyspnea on exertion was admitted to our hospital. A chest radiograph showed reticular opacities and volume loss in both lower lung fields. She was troubled with xerostomia and her laboratory test showed positive reaction for anti SS-A and SS-B antibody. Labial biopsy led to a diagnosis of primary Sjogren's syndrome (pSjS). Lung biopsy specimens obtained by video-assisted thoracoscopic surgery (VATS) revealed interstitial pneumonia. On the sixth postoperative day, hypoxemia acutely worsened and her chest radiograph showed widespread diffuse ground-glass attenuation. A diagnosis of acute exacerbation was made, and steroid and immunosuppressive therapy was started. In spite of intensive therapy, she died due to respiratory failure. We report a rare case of interstitial pneumonia with pSjS resulting in acute exacerbation.

A 29-year-old man was admitted with fever and anorexia. Radiographic examinations of the chest showed a localized peripheral non-segmental air-space consolidation in the right lower lobe. He had a history of exposure to parakeets, and psittacosis was diagnosed based on the elevated serum complement fixation titer against Chlamydia psittaci. The common radiographic finding of psittacosis is ground-glass attenuation radiating from the hilar areas. We report a rare case of psittacosis presenting a localized consolidation, clearly limited to the subpleural region of the lung.

We encountered two patients with clinically diagnosed idiopathic interstitial pneumonia with acute respiratory distress syndrome (ARDS). Both patients required mechanical ventilation within 24 hours after admission. We managed these patients using lower tidal volume ventilation (tidal volume: 5-6 ml/kg), antibiotics, sivelestat sodium hydrate and steroid pulse therapy followed by oral prednisolone therapy. Respiratory failure was relieved within 2 weeks following treatment There was no four-fold rise in screen viral titers and screening investigations of autoantibodies were negative. Based on these findings, we diagnosed these cases as having acute interstitial pneumonia (AIP). AIP represents a small subset of patients with ARDS without any associated or predisposing factor. Sivelestat sodium hydrate is an anti-neutrophil elastase inhibitor and is used for the treatment of acute lung injury (ALI)/acute respiratory distress syndrome (ARDS). Therefore, sivelestat sodium hydrate may be one of the treatment options for acute respiratory failure due to idiopathic interstitial pneumonia.

A 42-year-old woman was admitted with abnormal chest radiographs. Though interstitial pneumonia associated with dermatomyositis was diagnosed, her chest radiograph also revealed a narrowed trachea about 6 mm in diameter. Bronchoscopy showed that her trachea lacked a membranous posterior segment and O-shaped complete tracheal rings were present throughout the trachea, indicating congenital tracheal stenosis. Congenital tracheal stenosis is a rare disorder and is usually recognized in the first few weeks of life, but the patient had no history of dyspnea or recurrent pneumonia. This case suggests that among healthy people there are a very few who have asymptomatic congenital tracheal stenosis.

A 78-year-old man was referred to our hospital complaining of chronic productive cough. Physical examination revealed yellowish, thin nails and pretibial edema. A chest computed tomograph showed bilateral bronchiectasis. A sinus radiograph showed the findings of chronic sinusitis. From these findings, yellow nail syndrome was diagnosed. Long-term low-dose macrolide therapy with 400 mg/day clarithromycin was started and his symptoms began to gradually improve. However, complete resolution of his symptoms was not achieved and fiberoptic bronchoscopy was performed. Transbronchial biopsy specimen obtained from the right second carina showed bronchial asthma-like findings such as eosinophilic infiltration, thickening of the basement membrane, mucosal edema and goblet cell hyperplasia. Airflow reversibility was not detected. Thus a diagnosis of coexistence of yellow nail syndrome and eosinohilic bronchial disease was established. Further improvement of his symptoms was achieved by additional therapy with 800 microg/day budesonide and 100 microg/day salmeterol. To the best of our knowledge, this is the first report of a case of yellow nail syndrome associated with eosinophilic bronchial disease successfully treated with long-term low-dose macrolides and inhaled corticosteroids.

A 73-year-old female with interstitial pneumonia and systemic sclerosis was admitted with bloody sputum and fever. A chest radiograph showed bilateral infiltrative shadows superimposed on interstitial pneumonia. A chest computed tomograph also showed bilateral airspace consolidations and honeycomb formation. Mechanical ventilation was required because of respiratory failure. At intubation, fiberoptic bronchoscopy revealed bilateral bronchi to be filled with bloody secretions. Her serum myeloperoxidase-anti-neutrophil cytoplasmic antibody titer was slightly elevated. Steroid pulse therapy (1000 mg/day methylprednisolone for 3 days) and cyclophosphamide pulse therapy (500 mg cyclophosphamide) followed by 60 mg/day prednisolone was introduced on the basis of a diagnosis of diffuse alveolar hemorrhage. Her respiratory condition improved and chest infiltrative shadows disappeared and weaning from mechanical ventilation was successful. However, she died of ventilation insufficiency due to progression of her chronic respiratory failure. A necropsy specimen obtained from the left lung showed fibrosis, predominantly in peripheral areas of lobules, and accumulation of hemosiderin-laden macrophages, which findings were consistent with usual interstitial pneumonia and diffuse alveolar hemorrhage. A review of the world literature revealed 4 case reports of diffuse alveolar hemorrhage associated with interstitial pneumonia and systemic sclerosis. We should be aware of this rare but serious complication in the management of interstitial pneumonia associated with systemic sclerosis.

Bronchiolitis obliterans organizing pneumonia (BOOP) primed by radiation therapy for the breast cancer (BOOP-RT/BC) has been recognized recently. In this study, we reported 9 such cases and discussed steroid therapy. Mean radiation dose and interval from completion of radiation therapy to the appearance of BOOP-RT/BC were 47.3 Gy and 5.6 months respectively. BOOP-RT/BC recurrence was observed by 5 episodes in 3 of 9 cases (recurrence rate was 33%). Steroid therapy showed a remarkable improvement in both primary and recurrent disease, and no residual symptom or BOOP shadow was detectable at the end of the observation except for shadows due to radiation fibrosis. All but one patient were prescribed prednisolone, with a mean initial dose of 32.5 mg/day and a mean treatment duration of 46.7 weeks. One patient improved without treatment. No case suffered from respiratory failure which needed mechanical ventilation or was motile. Considering these findings and the fact that almost all patients are middle aged or senior women who are susceptible to osteoporosis, we conclude that steroid therapy for BOOP-RT/BC should be carefully considered.

There have been many reports studying the presentation for lipid antigen by CD1 molecules on dendritic cells (DC), mainly in the infection of acid-fast bacilli. But little is known about the expression of CD1 molecules in sarcoidosis. In this study, we analyzed the expression of CD1 molecules by immunohistochemical stain with monoclonal anti-CD1a, CD1b and CD1c antibody for the specimens of nine sarcoidosis patients (sarcoidosis group) and seven control cases (control group). Aggregation of CD1 positive cells was present adjacent to granulomas in five cases of the sarcoidosis group, but was absent in all cases of the control group. There were no differences in the results of laboratory findings or disease activity between CD1-positive and negative cases in the sarcoidosis group. These data suggest that the presentation of lipid antigens mediated by CD1 molecules on DC is involved in granuloma formation in sarcoidosis.

A 15-year-old man was incidentally found to have abnormal shadows in bilateral lungs on a chest X-ray film obtained at a school medical examination, and was referred to our hospital for a close investigation. Several lymph nodes were palpable in his cervical region. Chest X-ray and CT showed multiple and various sized nodules up to 20 mm in diameter in bilateral lung field. Abdominal CT showed multiple calcified lesions and low density areas in the liver. We performed video-assisted thoracoscopic lung biopsy, and diagnosed this case histologically as pulmonary epithelioid hemangioendothelioma. The tumor cells showed immunohistochemically positive staining for Factor VIII-related antigen that was one of endothelial cell marker. He has been followed up for 2 years and 11 months after the surgical lung biopsy without specific medication and remains asymptomatic at the time of writing this paper.

A 47-year-old woman was admitted to our hospital because of dry cough, fever, and subacute, progressive dyspnea. Chest radiography and computed tomography showed ground glass opacities in the lower lung fields. We suspected pneumonia caused by atypical pathogens and administered antibiotics, but they had no effect at all. Histopathologic findings from a transbronchial lung biopsy (TBLB) included intensive infiltration of mononuclear cells and edema on the alveolar wall with no evidence of fibrosis, fibroblasts, hyaline membrane, or granuloma. On the basis of these findings, we suspected interstitial pneumonia, but a surgical lung biopsy was not possible because the patient would not give her consent. After TBLB, corticosteroid was administered repetitively, but dyspnea was deteriorating as the ground glass opacities became more widespread, and tractional bronchiectasis appeared throughout the lung fields. Therefore, we decided to administer cyclophosphamide (CPA). This was very effective: all of her symptoms improved and the ground glass opacities and tractional bronchiectasis disappeared. Though we tapered and then discontinued corticosteroids a few months after CPA, there was no recurrence whatever. No signs suggesting the association of collagen vascular diseases were detected. The effectiveness of CPA in interstitial pneumonia associated with collagen vascular disease is occasionally reported, but the effect on idiopathic interstitial pneumonia, especially in acute and subacute progressive cases, is rarely reported. We think this is an interesting case to consider the availability of CPA in idiopathic interstitial pneumonia with subacute progression.

Recently, cyclosporin has been reported to be a promising drug for the treatment of interstitial pneumonia. Monitoring of the serum cyclosporin concentration is important for the safety and efficacy of treatment. We measured the concentrations of this drug just before (C0) and 2 hours after (C2) administration, and the area under the concentration-time curve from the start of administration for 5 hours (AUC 0-5) in 58 patients. We found that C2 has the strongest correlation with AUC 0-5, which indicates the efficacy of cyclosporin. In 11 cases of interstitial pneumonia, 5 showed deterioration despite cyclosporin treatment. Three of those 5 cases had low C2 and AUC 0-5 levels, indicating that they were low absorbers and slow absorbers, which may be associated with a poor response. Therefore, the monitoring of the cyclosporin concentration is important especially in progressive cases of interstitial pneumonia that deteriorate despite cyclosporin treatment.

We retrospectively investigated the efficacy of regimens including clarithromycin (CAM) in 129 patients with Mycobacterium avium complex (MAC) pulmonary disease and their outcomes. None of the patients suffered from acquired immunodeficiency syndrome. All were observed for over 12 months. We studied the factors that may affect sputum conversion and fatal outcomes by logistic regression analysis. The results indicated that the presence of either cavitation or bronchiectasis was significantly correlated with persistent MAC-positive culture results in sputum (Odds ratio, 4.71, 95%; CL, 1.21-18.5; P<0.05). Regimens including antituberculous drugs and CAM were administered to 118 patients, 11 of whom received CAM alone because of the adverse events of antituberculous agents. There was no difference in sputum conversion or mortality between the two groups, suggesting that the pattern of drug combination should be further investigated.

A 52-year-old woman was hospitalized because of severe cough in August 1994. She had engaged in culturing roses in greenhouses since 1968, and had developed a cough during the summer of 1990. Chest radiography showed diffuse ground-glass opacity in both lung fields, and she suffered from hypoxemia (PaO2 = 45.6 torr) while breathing room air. The lymphocyte count in the bronchoalveolar lavage fluid was increased, and transbronchial lung biopsy specimens showed lymphocyte alveolitis in the alveolar spaces. After admission, the patient's symptoms improved rapidly without medication. However, on her return to work, the cough and hypoxemia reappeared. In her rose culture, she had used Rockwool, and Aspergillus niger was detected predominantly in the Rockwool. Precipitins against the extracts of Aspergillus niger were detected with the double immunodiffusion test and the inhalation provocation test yielded clinical symptoms. Our diagnosis was hypersensitivity pneumonitis caused by Aspergillus niger.

A 51-year-old woman was referred to our hospital with a complaint of disturbance in vision. Ophthalmologic examination revealed multiple choroidal tumors. High-resolution CT showed a nodular shadow in the left lower lobe. Transbronchial biopsy and right supraclavicular lymph node biopsy specimens showed a poorly-differentiated adenocarcinoma. We concluded that the choroidal tumors had metastasized from the lung. Combined chemotherapy (CDDP + CPT-11) followed by irradiation of both eyes and brain were performed. Nevertheless, she died 6 months after the initial presentation. It is important to notice ophthalmologic symptoms because lung cancer may metastasize to the choroids.

Nonspecific interstital pneumonia (NSIP) is associated with an excellent response to steroidal therapy. However, it was reported that some cases of NSIP might have an unfavorable outcome in spite of aggressive therapy. In this study, we explored the prognosis for 23 patients with NSIP and examined matched groups of 18 patients with treatment-responsive or naturally recovered idiopathic NSIP, and 5 patients with idiopathic NSIP who fared poorly despite therapy. The patients who had poor prognoses were, significantly, all non-smoking women. Histologic examination revealed that the cases with progressive, idiopathic NSIP were in all cases of the fibrotic type. By contrast, only 39% of those with a good prognosis, were fibrotic. No significant differences in CT appearance were noted between the two cohorts. These findings provide a potential clinical marker of therapy outcome of in idiopathic NSIP.

We report two cases of intralobar pulmonary sequestration associated with nontuberculous mycobacterial infection. Case 1: A 28-year-old woman was hospitalized because of cough. Chest CT demonstrated multi-cystic lesions with an air-fluid level in the right lower lobe. Case 2: A 25-year-old woman was hospitalized because of fever. Chest CT demonstrated homogeneous consolidation in the right lower lobe. In both cases, aortography showed an anomalous artery supplying the right basal segment, and pulmonary sequestration was diagnosed. Furthermore, Mycobacterium avium was detected in the bronchial lavage, and a right lower lung lobectomy was performed after chemotherapy. Mycobacterium avium was cultured from the fluid in the sequestrated lung, and histological findings of the resected lobe showed epithelioid cell granulomas. We diagnosed these cases as having pulmonary sequestrations associated with nontuberculous mycobacterial infection. We concluded that pulmonary sequestration should be considered an underlying disease of secondary nontuberculous mycobacterial infections in young patients.

A 76-year-old man without symptoms was admitted to our hospital for investigation of an abnormal chest shadow in 1994. His chest radiograph showed a nodular shadow in the lingual lobe. Segmentectomy was performed and the histological diagnosis was MALT (mucosa-associated lymphoid tissue) lymphoma. In June 2001, there were no abnormal shadows on the chest radiograph or in 10 mm slice CT. However, high-resolution CT with 2-mm slice thickness revealed diffuse micronodular shadows in both lungs. A relapse of MALT lymphoma was diagnosed by immunohistochemical analysis of tissue specimens obtained by transbronchial lung biopsy. It was observed that the patterns of radiological findings in primary pulmonary MALT lymphoma cases may differ between the initial state and a relapse.

A 52-year-old man was hospitalized because of recurrent fever, dyspnea and cough in February 2002. He had worked in a Eringi (Pleurotus eryngii) mushroom factory since 1996. Chest radiography showed diffuse fine nodular shadows. Chest computed tomography demonstrated centrilobular nodules and increased attenuation in both lungs. The patient suffered from hypoxemia (PaO2 = 65 torr) while breathing room air. The lymphocyte count in the bronchoalveolar lavage fluid was increased, and transbronchial lung biopsy specimens showed lymphocyte alveolitis with epithelioid cell granulomas in the alveolar spaces. After admission, the patient's symptoms improved rapidly without medication. However, on his return to work, fever and hypoxemia appeared again. The lymphocyte stimulating test was positive against extracts of Eringi spores. Precipitins against the extracts of Eringi spores were detected by the double immunodiffusion test. Our diagnosis was hypersensitivity pneumonitis (HP) caused by Eringi spores. In Japan, more than 30 cases of HP induced by mushroom spores have been reported. It is a matter of great urgency to prevent the occurrence of occupational HP in mushroom factories.

A 55-year-old man was admitted to our hospital with dyspnea on effort and sclerodactyly. The chest radiograph showed reticulonodular shadows in both lower lung fields. A diagnosis of systemic sclerosis was made based on the characteristic skin lesions, interstitial pneumonia and the positivity for anti-Scl-70 antibody. Surgical biopsies were performed, which showed various features characteristic of organizing pneumonia and non-specific interstitial pneumonia. Prednisolone and cyclophosphamide were given to the patient, but clinical symptoms and chest radiographic findings worsened. After cyclophosphamide was replaced with cyclosporine, his symptoms, chest radiographic findings and pulmonary function improved and stabilized. It is suggested that cyclosporine is useful for the treatment of progressive interstitial pneumonia associated with systemic sclerosis.

A 47-year-old man was admitted to our hospital because of progressive dyspnea and cough. Physical examination and chest radiographs showed the signs of cor pulmonale. A lung scan using perfused radionuclide revealed multiple peripheral perfusion defects and catheterization of the right heart showed severe pulmonary hypertension. A diagnosis of severe pulmonary embolism was made. Despite intensive care with anti-coagulation therapy, the patient died on the third-hospital day. Autopsy disclosed gastric cancer in the pylorus with metastases to the regional lymph nodes. There were no macroscopic pulmonary artery emboli or parenchymal lesions, but more than 60% of the small arteries and arterioles were occluded by casts of tumor cells. Cor pulmonale due to a pulmonary tumor embolism is a rare complication of cancer. This case is particularly unusual because the embolus-caused cor pulmonale was the initial manifestation of clinically occult, but pathologically advanced, gastric cancer.

A 50-year-old woman with abnormal shadows in her chest radiograph was admitted for a more detailed examination in April 2000. A chest CT scan showed infiltrates surrounded by ground-glass opacities in the right upper lobe, and ground-glass opacities with partial infiltrative changes in the left lower lobe. A diagnosis of adenocarcinoma like bronchioloalveolar carcinoma (BAC) was made for the lesion in the right upper lobe by means of a transbronchial biopsy. However, the specimen from the left lower lobe showed nonspecific changes, and bronchiolitis obliterans organizing pneumonia (BOOP) or a metastatic lesion from the BAC was suspected. We prescribed steroids for the short term. Since the lesion in the left lobe disappeared, we performed a right upper lobectomy. In cases of primary lung carcinoma, BOOP in the opposite lung is rare and it was difficult to decide on the operability. We concluded that steroid therapy is useful for distinguishing between inflammatory and tumorous lesions in cases in which a detailed examination is difficult.

Chronic hypersensitivity pneumonitis (CHP) has a poor prognosis because of the difficulties in its diagnosis and treatment. Recently, we have encountered six cases of CHP (4 patients with the home-related type, a patient with bird fancier's lung, and one with flour-induced CHP), and we examined the clinical characteristics of these cases. Environmental provocation testing has been considered useful for diagnosing HP, but all patients gave negative results in short-term environmental exposure tests performed routinely for the diagnosis of HP. However, 5 patients had a positive response in long-term environmental exposure tests. Two patients died of respiratory failure after ten years' observation despite improvement of the causative environment, and were given steroid therapy. Radiographically and pathologically, the process of progressive and irreversible lesions were recognized in our series of CHP patients. The diagnosis of CHP should be carried out on the basis of the comprehensive findings including the detailed history, clinical course, radiography, and histology.

A 51-year-old man with myelodysplastic syndrome (MDS) was admitted to our hospital because of dyspnea on exertion in December 1999. Chest radiography showed ground-glass shadows in the middle and lower fields of both lungs, and chest computed tomography revealed a typical "crazy paving appearance". The bronchoalveolar lavage fluid was milky in appearance, and so secondary pulmonary alveolar proteinosis associated with MDS was diagnosed. Because there was no need to treat his MDS, we twice performed whole-lung lavage under general anesthesia in January and February 2000. The treatments were effective, and his abnormal chest radiography findings, laboratory data and pulmonary function were normalized. This was a rare case of secondary pulmonary alveolar proteinosis associated with MDS successfully treated with whole-lung lavage.

A 42-year old man was admitted to our hospital for evaluation of pleural effusion in the right hemithorax. He had been treated for spastic paraplegia with dantrolene sodium for 28 months. The pleural fluid consisted of sterile exudates with a very high eosinophil count, and peripheral blood eosinophilia was noted. Thoracoscopy revealed no apparent abnormalities and a pleural biopsy specimen showed only non-specific inflammation. Two weeks after discontinuing dantrolene therapy, the pleural effusion disappeared. The toxicity of dantrolene in patients with pleural effusion must be taken into consideration when long-term dantrolene therapy is given.