Nihon Kokyuki Gakkai zasshi = the journal of the Japanese Respiratory Society最新文献

A 72-year-old man presented to our hospital with fatigue and anemia. Chest CT showed multiple nodular shadows. We first suspected lung cancer and multiple metastatic lesions because some nodules had spiculation. However, PET-CT revealed the small intestine, thyroid and rib as well as these nodules to be positive for FDG uptake, suggesting malignant lymphoma and lung involvement. For diagnosis, lung biopsy by video-assisted thoracic surgery (VATS) was performed. Pathologic examination of the lung biopsy specimen showed diffuse large B-cell lymphoma. We diagnosed secondary pulmonary malignant lymphoma.

A 68-year-old woman was admitted to our institution's respiratory section because of dyspnea on effort in January, 2007. She had previously received a diagnosis of Sjögren's syndrome because of dryness in her eyes in 1991. Chest radiography and chest CT in 2001 revealed diffuse multiple cystic lesions in both lungs which had progressed gradually for 6 years. Biopsy specimens obtained by video-assisted thoracoscopy showed lymphoid hyperplasia with follicular bronchiolitis and lymphocytic alveolitis. Narrowing of the small airways and obstructive lung disease with multiple bullae were observed and we suspected them to be related to peribronchiolar lymphocytic infiltration. These were lung involvements associated with Sjögren's syndrome. The patient's cystic lesions gradually worsened despite the administration of corticosteroid and cyclophosphamide. Cystic lesions in Sjögren's syndrome may be a treatment-resistant finding.

A 54-year-old man was admitted to Saga University hospital with dyspnea on effort and a sensation of pressure in the chest. Chest CT images showed a low-density mass in the mediastinum surrounding the carina and left hilus, causing narrowing of both the left pulmonary artery and left main bronchus. The pathological findings from a surgical biopsy showed markedly fibrotic tissue with lymphocytes and plasmacytes, and we diagnosed idiopathic fibrosing mediastinitis, stage II. Oral glucocorticoid treatment of 30 mg/day prednisolone reduced the mass and improved the narrowing of the left pulmonary artery and left main bronchus. The patient was given low-dosage glucocorticoids as maintenance treatment. Previous reports indicated that idiopathic fibrosing mediastinitis with severe tissue fibrosis is difficult to control with glucocorticoid monotherapy. Here, we report a case of idiopathic fibrosing mediastinitis that was effectively treated with glucocorticoids.

Background: To investigate the relationship between the prognosis of chronic interstitial pneumonia (IP) and its comorbidities, we conducted a retrospective study for clinically and radiologically diagnosed IP. We assessed comorbidities by using the Charlson Comorbidity Index (CCI).

Methods: We classified 224 patients given clinical diagnoses of chronic IP (excluding the patients who had clear causes such as collagen disease, infection, drugs or radiation) in our institution between April 2000 and June 2010, into 2 groups; those with clinical diagnoses of idiopathic pulmonary fibrosis (IPF:108 cases) and those with other chronic IP but without honeycomb lung (116 cases); and analyzed their backgrounds and comorbidities. We also classified them into survival and non-survival groups to assess their prognostic factors.

Results: Although the smoking status of patients with clinically diagnosed IPF was higher, and SpO2 was lower than those with other chronic IP without honeycomb lung, the mean age, comorbidities and CCI did not differ between them. The 5-year overall survival of the clinically-diagnosed IPF group was lower than that of the other chronic IP without honeycomb lung group (50.8% vs. 76.3%, p<0.01). In cases of other chronic IP without honeycomb lung, the CCI of non-survival cases was higher than that of survival cases (4.05 vs. 2.47, p<0.01), although patient backgrounds did not differ between survival and non-survival cases in those with clinically diagnosed IPF (CCI : 2.32 vs. 2.98, p = 0.70).

Conclusions: Our analysis revealed the possibility that comorbidities and CCI were prognostic factors in other chronic IP cases without honeycomb lung, although the prognosis of IPF was not affected by their comorbidity.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital disorder characterized by varicose veins, cutaneous hemangiomas, hypertrophy of soft tissue and bone and arteriovenous malformations. We present a case of a 43-year-old man with KTWS. He experienced progressive pulmonary hypertension due to recurrent pulmonary embolism, which developed despite adequate anticoagulation. This case report suggests that patients with KTWS need more aggressive management and treatment of their thromboembolitic state and pulmonary hypertension.

A 53-year-old man presented to our hospital with cough in August 2004. Chest computed tomography showed an anterior mediastinal tumor, centrilobular nodules and mild bronchiectasis. Centrilobular nodules were improved by the administration of clarithromycin, and we resected an anterior mediastinal tumor (thymoma). Partial lung resection was also performed, and a diagnosis of follicular bronchiolitis was established. The number of centrilobular nodules increased after cessation of the clarithromycin, and its administration was reinitiated; however, the patient's bronchiectasis and airway obstruction worsened. Steroid administration was begun in January 2010; thereafter, pulmonary function, bronchial wall thickness, cough and shortness of breath improved. Since April 2004, the patient has experienced 12 episodes of airway infection. Careful attention should be paid to recurrent airway infection, progressive bronchiectasis and airway obstruction in patients with follicular bronchiectasis.

A high-dose administration of inhaled corticosteroid is effective in the majority of patients with bronchial asthma, but is often difficult to attain sufficient control in certain subsets of patients. Omalizumab has recently emerged as a promising drug for bronchial asthma. To assess its add-on effect we administered omalizumab to patients with uncontrolled atopic asthma for more than 16 weeks and gave them questionnaires. The study population comprised 9 patients with frequent asthmatic symptoms despite the administration of high-dose inhaled corticosteroid and other disease controllers. We scored disease control using the Asthma Health Questionnaire-33-Japan and the Asthma Control Test, and evaluated the frequencies of short-acting beta2-agonist use for rescue and drip infusion of theophyllines and/or systemic steroids in a retrospective fashion. Asthmatic scores were significantly improved after 16 weeks of omalizumab therapy. The frequencies of reliever use and drip infusion were also decreased. These trends were present even in patients in whom no aeroallergen-specific IgE antibodies were detected. No statistically significant side effects were observed. Our study confirmed the add-on effect of omalizumab based on evaluation by simple questionnaires. Further studies are needed to clarify whether omalizumab therapy is suitable for patients without specific IgE antibodies.

A 52-year-old man was given a diagnosis of ulcerative colitis and treated with mesalazine for 7.5 years. However, the unusually long administration of mesalazine induced lung injury and the patient complained of a dry cough and dyspnea on limited exertion. Infiltrative shadows were observed in bilateral lung fields on a chest radiograph and computed tomographic images. The histological findings obtained by transbronchial biopsy and bronchoalveolar lavage showed organizing pneumonia. His drug-induced lymphocyte stimulation test (DLST) for mesalazine was positive. Improvements in his clinical symptoms and radiographic abnormalities occurred spontaneously after the discontinuation of mesalazine. This case indicates that the long-term administration of mesalazine may lead to an adverse pulmonary reaction.

We analyzed the routes of draining veins in 7 cases of intralobar pulmonary sequestration (ILS) and 6 cases of bronchial atresia (BA) by multidetector CT and on 3D reconstructed images. In 2 cases of ILS with systemic venous drainage and 4 of 5 cases with pulmonary venous drainage, the primary veins penetrated the abnormal lungs. In another case, the main vein bypassed the border of the lung, but a small number of branches joined from the normal lung. In all cases of BA, draining veins distributed outside the abnormal lung. The finding that ILS has its own draining veins supports the theory of its congenital origin from an accessory lung bud.

The prognosis of idiopathic pulmonary fibrosis (IPF) is poor, and it deteriorates when it is complicated with pulmonary hypertension (PH). Forced vital capacity (FVC) is a useful parameter for evaluating the disease status of interstitial pneumonia (IP). However, in patients with IP complicated with emphysema, the disease severity can be overlooked because of relatively well preserved FVC. We investigated the correlation between the maximum pressure gradient (PG) in the tricuspid valve using echocardiographic measurements and pulmonary function tests in patients with IP without emphysema and in those with IP with emphysema. There was an inverse correlation between PG and %FVC in patients with IP without emphysema. However, the above inverse correlation between PG and %FVC mentioned above disappeared when analyzed in the whole cohort of patients (n = 42) consisting of IP without emphysema (n = 35) and IP with emphysema (n = 7). Patients with IP without emphysema did not show a correlation between PG and %FEV1, but when analyzed using the whole cohort of patients an inverse correlation between PG and %FEV1 was observed (p<0.05). In clinical practice, not only FVC, but also %FEV1 is a valuable parameter in investigating the complication of emphysema and PH in patients with chronic idiopathic interstitial pneumonia.