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Background We are occasionally presented with patients with unclassifiable interstitial pneumonia of unknown etiology. Idiopathic pulmonary upper lobe fibrosis (IPUF) does not fit any of the currently defined subsets of idiopathic interstitial pneumonias (IIPs). This study was performed to examine clinical, functional, and pathological characteristics of IPUF. Methods We present 9 cases of histologically confirmed IPUF. The clinical and histological characteristics of the 9 patients were evaluated. The baseline respiratory function of all patients was measured. There were 7 patients whose forced vital capacity (FVC) had been monitored for at least a year who were selected to quantify the yearly decline in FVC. Results All patients were slender, with a body mass index of 16.0–19.8kg/m 2 . Seven patients had a history of pneumothorax. Six patients died 1.8 to 5.7 years after the onset of the first symptoms. Fundamental histological features were intraalveolar collagen deposition and densely packed elastic fibers in the subpleural areas. These findings are the same as those seen in pleuroparenchymal fibroelastosis. However, the visceral pleura was thickened with dense collagen in only 2 patients, and pleural thickening was localized, if present, in the remaining 7 patients. Ventilatory impairment was also a characteristic. The time course decline of FVC was rapid and almost linear. The median yearly decline in FVC was −20.3% (range, −7.7% to −26.5%), which was more rapid than that reported for chronic fibrosing interstitial pneumonias such as idiopathic pulmonary fibrosis. Conclusions IPUF is a unique pulmonary fibrosis that results in rapid deterioration of ventilatory function and poor prognosis.

背景. Gefitinibは化学療法による既治療が奏効しなかった肺腺癌患者に投与されるが, 下痢などの有害事象はこれらの患者にとって深刻な問題となる. 症例. 71歳女性. 2002年2月に肺腺癌 (臨床病期IIIB) と診断した. プラチナ製剤を含む化学療法を行ったが無効であったので, gefitinib 250 mg/日の投与を行った. 重篤な下痢が出現したために塩酸ロペラミドを投与し, 7日間休薬した後, 隔日投与にて治療を再開した. その結果, 下痢は止まり腫瘍の縮小率は44% (RECIST) に達しPRと判定した. 結論. 現在までに, 国内外ともに隔日投与により, 重篤な副作用を回避し臨床効果をあげられた報告はなく, 今後は隔日投与の体内動態を再検討し, 他の内服方法と比較することで臨床効果のみならず, 有益な経済効果をもたらす可能性があると考えられた.

We investigated the effects of a novel oral neutrophil elastase inhibitor (ONO-6818) on acute lung injury and pulmonary emphysema induced by human neutrophil elastase (HNE). Young male Wistar rats were divided into four treatment groups: (1) control group (saline); (2) HNE group (HNE 200 U + 0.5% carboxymethyl-cellulose [solution for ONO-6818]); (3) low-dose ONO-6818 group (HNE 200 U + ONO-6818 10 mg/kg); and (4) high-dose ONO-6818 group (HNE 200 U + ONO-6818 100 mg/kg). Saline and HNE were applied via the trachea using a microsprayer. ONO-6818 was administered orally 1 hour before HNE application. Six hours after HNE application, neutrophil counts and hemoglobin concentration in bronchoalveolar lavage fluid and lung tissue myeloperoxidase activity were determined. Eight weeks after the application, FRC, TLC, lung compliance, and mean linear intercept were estimated. ONO-6818 attenuated dose-dependently HNE-induced increases in lung myeloperoxidase activity, hemoglobin, and neutrophil count in bronchoalveolar lavage fluid. Furthermore, it significantly attenuated HNE-induced increases in FRC, TLC, lung compliance, and mean linear intercept. ONO-6818 inhibited acute lung injury induced by HNE by minimizing lung hemorrhage and accumulation of neutrophils in the lung. ONO-6818 also inhibited the development of HNE-induced emphysematous changes including lung hyperinflation, degradation of elastic recoil, and airspace enlargement.

We report a case of pulmonary aspergillosis in lung transplant recipient who was successfully treated with inhalation administration of anti-fungal agent. The case was 33-year-old female. Two years ago, she had received lung transplant because of lymphangioleiomyomatosis. One year ago, she had diagnosed of pulmonary aspergillosis and successfully treated with micafungin and itraconazole. Then she had been continuous administered with itraconazole. In June 20xx, she had nausea and vomiting and was diagnosed of viral enteritis. Although abdominal symptoms were relieved, ground glass opacity was discovered in her right lung. Bronchoscopic examination revealed ulceration of bronchus with white necrotic substance. Laboratory culture test demonstrated Aspergillus spp. Finally she was diagnosed of recurrent pulmonary aspergillosis. First, she was treated with intravascular administration of micafungin. Then, inhalation administration of liposomal amphotericin B was changed. Ground glass opacity and bronchial region of pulmonary aspergillosis was improved. Thereafter, inhalation of amphotericin B was continued and no recurrence of pulmonary aspergillosis has been found. Inhalation of anti-fungal agent could be an option for pulmonary aspergillosis.

A 18-year-old man complaining of remittent fever and nonproductive cough visited a nearby clinic. He did not recover despite treatment of oral azithromycin. We admitted him because his chest radiograph showed consolidation in the left upper lung field. We diagnosed his pneumonia as co-infection by non-bacterial and bacterial pathogens, and initiated treatment with intravenous ampicillin and oral clarithromycin. On the 3rd day after admission his symptom had not improved, so his treatment was changed to intravenous panipenem/betamipron and erythromycin. Ciprofloxacin was administered intravenously because consolidative shadows with atelectasis increased on the chest radiograph on the 6th day. Clinical symptoms such as fever, CRP and chest radiograph findings were rapidly improved after the start of ciprofloxacin treatment. He was discharged on the 22nd hospital day. Since serum antibody titer against Mycoplasma pneumoniae was elevated to x 20,480 on the 13th hospital day, it is confirmed that causative pathogen was macrolide-ineffective Mycoplasma Pneumoniae.

A 79-year old woman underwent total gastrectomy under the diagnosis of gastric cancer in Feb. 2003. In the beginning of Jan. 2005, she noticed hemosputum and was admitted to our hospital. Chest radiograph and CT disclosed bilateral upper lobe-dominant nodular opacities in the subpleural areas and ground-glass opacities in right S6. Transbronchial lung biopsy was performed, but no useful information for the diagnosis was obtained. Ziehl-Neelsen stain was negative for the smear of the sputum at admission, but weakly stained acid-fast bacilli were grown in the MGIT culture. By the analysis of mycolic acid and menaquinone of the cell membrane, the bacilli were identified as Tsukamurella. Since she was asymptomatic and repeated sputum examination revealed negative bacilli, she has been observed at the outpatient clinic without any treatment.

An autopsy case of fibrotic non-specific interstitial pneumonia (NSIP) is herein reported. A 54-year-old woman was admitted to our hospital because of dry cough and fever that had continued for a month. Her chest radiograph showed diffuse reticular shadows in both lower lung fields. Analyses of bronchoalveolar lavage fluid (BALF) showed an increase in the percentage of lymphocytes and a decrease in CD4/CD8 ratio. Video-assisted thoracoscopic (VATS) lung biopsy revealed that she had fibrotic NSIP. She was treated with corticosteroid with a transient increase in vital capacity, but her condition gradually deteriorated, associated with a decrease in lymphocytes and an increase in CD4/CD8 ratio shown by repeated measurement of BALF. She died 6 years after the diagnosis. The autopsied lungs showed diffuse consolidated lesions predominantly in both lower lung fields, without honeycombing. Histologically, the lung parenchyma was diffusely involved with homogeneous fibrosis, compatible with fibrotic NSIP. However, mononuclear cell infiltration was less severe, and collagen deposition was more extensive than shown by the VATS specimen. There is a possibility that the CD4/CD8 ratio in BALF may reflect the severity of fibrosis in the lung parenchyma. Histological differences between autopsy and biopsy specimens in this case could help to elucidate the natural course of fibrotic NSIP.

A 17 year-old youth presented with swelling of both sides of neck after a fight with a friend. He had been sick with an upper respiratory tract infection for a few days with frequent coughing. Chest radiography showed subcutaneous and mediastinal emphysema. Neck CT at the level of C7 showed air around the trachea, extending to the subcutaneous tissue and the epidural space through the intervertebral foramen. His height was 180 cm and his weight 55 kg, and he had a 181 cm arm span. He had scoliosis and arachnodactyly, and ultrasonic cardiography demonstrated mitral and tricuspid regurgitation. These findings agreed partially with the clinical criteria of Marfan syndrome. Thus, forme fruste of Marfan syndrome was suspected. A rapid rise of airway pressure induced by a coughing attack and loud shouting during the fight probably caused the laceration of the connective tissue in the airway, resulting in mediastinal and epidural emphysema. In this case report, CT at the C7 level satisfactorily identified air in the mediastinum extending to the epidural space through intervertebral foramen.