[A case of follicular bronchiolitis in which progressive bronchiectasis developed over 7 years].

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.

[Eight cases of idiopathic pulmonary alveolar proteinosis followed up for more than 4 years].

BACKGROUND: Few reports exist regarding the long-term clinical course of idiopathic pulmonary alveolar proteinosis. PURPOSE AND METHODS: We retrospectively studied the clinical courses of 8 patients. The patients were 4 men and 4 women aged 48.4 +/- 14.0 years (mean +/- SD) with idiopathic pulmonary alveolar proteinosis followed up for a minimum of more than 4 years. Mean follow-up was 13 years and 6 months (4-22 years and 9 months). RESULTS: There were 15 instances of whole-lung lavage and/or bronchoalveolar lavage in 7 patients, and lavage was effective 40% of cases. Spontaneous improvement occurred 16 times and exacerbation occurred 7 times in 8 patients. On chest X-ray films, the shadows disappeared in 5 of 8 (62.5%) patients, and in 4 of 5 patients, shadows disappeared 5 or more years after diagnosis of pulmonary alveolar proteinosis. Pulmonary shadows have not disappeared in the remaining 3 patients at the time of writing but have shown improvement compared with first presentation. There were no deaths. CONCLUSION: As indicated in previous reports, the long-term outcome of idiopathic pulmonary alveolar proteinosis appears to be good.

[Comparison between pandemic H1N1 2009 influenza pneumonia and seasonal influenza pneumonia in adults].

We compared 126 cases of seasonal influenza pneumonia (seasonal flu) reported between January, 1996 and March, 2009, with 10 cases of laboratory-confirmed pandemic influenza (H1N1) 2009 influenza virus pneumonia (novel flu), based on clinical condition, computed tomography (CT) findings, severity, treatment, and prognosis, to clarify the characteristics of this novel flu. The mean age of subjects was 52.4 years in the novel flu group and 64 years in the seasonal flu group, and novel flu patients were younger than seasonal flu patients. Seasonal flu patients had more underlying diseases than did novel flu patients. The median duration from illness onset to hospitalization was 4 days in both groups. Primary viral pneumonia was present in 70% of novel flu cases and 31% of seasonal flu cases. The proportion of primary virus pneumonia was higher in novel flu patients, and the disease severity of the seasonal flu group was more severe than that of the novel flu group. White blood cell and lymphocyte counts were lower in novel flu patients, and chest CT images showed bilateral shadows and pure ground-glass opacities more frequently in the novel flu cases. There were no differences in treatment, number of days required for the fever to subside, or mortality between the groups.

[Clinical study of pneumocystis pneumonia in patients with rheumatoid arthritis].

We reviewed case of pneumocystis pneumonia (PCP) with rheumatoid arthritis. We administered the antirheumatic drug methotrexate (MTX) at the time of to 13 patients, corticosteroids to 11 patients and a tumor necrosis factor (TNF) inhibitor to 3 patients. Treatment for PCP was started on admission in all cases. We administered adrenocorticosteroids to all 13 patients with a PaO2 level < 70 Torr. Three patients were under respiratory management, and 4 patients died. By univariate analysis, prognostic indicators of death were: presence of acute respiratory distress syndrome (ARDS), peripheral blood neutrophil/lymphocyte ratio, serum albumin value, serum beta-D-glucan value, and AaDO2 and PaO2/FiO2 ratios. Readministration of a TNF inhibitor in 2 patients and MTX in 3 patients was possible after PCP remission. Even though we began treatment for PCP on the day of admission, 25% of patients died. PCP may occur in patients who are given MTX or a TNF inhibitor or both, and the clinician should endeavor to detect its onset as early as possible. Elucidation of the prognostic indicators of recovery may require multivariate analysis of many cases.

[A single-center prospective study of viral infection in community-acquired pneumonia in adults].

From July, 2008 to March, 2009, 125 adults with community-acquired pneumonia (CAP) who were admitted to our hospital were retrospectively investigated to elucidate the characteristics of viral infection in adult CAP in Japan. Nasopharyngeal swabs for real-time polymerase chain reaction for 7 types of influenza virus, rhinovirus, respiratory synctial virus, human metapneumovirus, parainfluenza virus, coronavirus, and enterovirus were obtained. Diagnoses of viral infections were established according to positive results in real-time polymerase chain reaction and influenza rapid diagnostic testing, and based on a fourfold increase in antibody titer of influenza virus antibody in paired sera. Overall, a pathogen was identified in 74 patients (59.8%). Of these pathogens, 47 (37.6%) were bacterial, 17 (13.6%) were viral, and 10 (8.0%) were mixed virus and bacterial infection. Influenza virus (n = 12; 9.6%), rhinovirus (n = 8; 6.4%), respiratory syncytial virus (n = 8; 6.4%), and parainfluenza virus (n = 6; 4.8%) were detected. Adenovirus, coronavirus or enterovirus was not detected. Sore throat was more frequently found in patients with viral pneumonia than in those with non-viral pneumonia. Higher age and pneumococcal pneumonia were factors which contributed to severity in the present cases. It is difficult to distinguish viral pneumonia from non-viral pneumonia by clinical findings, and there were few clinically meaningful differences in presentation and severity, and no differences in severity or outcomes according to either the presence or absence of viral infection. Further studies are needed to clarify the possible significance of viral infection in CAP.

[A case of pulmonary alveolar proteinosis which initially deteriorated rapidly with exacerbation of pulmonary nocardiosis, responded promptly to treatment of the pulmonary nocardiosis].

A 37-year-old man was admitted with complaints of continuous cough and sputum production for 1 month. Computed tomography (CT) of the chest revealed a solitary mass with a cavity in the apex of the left lung and bilateral ground-glass opacities (GGO). Thereafter, the patient complained of fever, and an increase in the mass shadow size and expansion of the ground-glass opacities were observed on serial CT. The patient was given diagnoses of pulmonary nocardiosis and pulmonary alveolar proteinosis by bronchoscopic examination. Serum anti-GM-CSF antibody tests were positive. On the basis of these findings, we diagnosed autoimmune pulmonary alveolar proteinosis. After beginning antituberculosis drugs and antibiotics, the tumor shadow and GGO reduced. The pulmonary alveolar proteinosis rapidly worsened on exacerbation of the pulmonary nocardiosis, but prompt overall improvement was obtained after treating the latter. We believe this to be a valuable case for examining the time progression of autoimmune pulmonary alveolar protein syndrome, because of the clinical course of the exacerbation, and the improvement in the pulmonary alveolar proteinosis after treatment of pulmonary nocardiosis.

Changes in lung function and health status in patients with COPD treated with tiotropium or salmeterol plus fluticasone.

BACKGROUND AND OBJECTIVE: The effects of tiotropium, a long-acting anticholinergic drug, were compared with those of the combination of salmeterol, a long-acting beta(2)-agonist, and fluticasone, an inhaled corticosteroid, in patients with COPD. METHODS: A 4-month, randomized, open cross-over study of tiotropium, 18 microg once daily, versus salmeterol, 50 microg, plus fluticasone, 200 microg, twice daily, was conducted in patients with COPD. Efficacy was assessed by spirometry and responses to the St George's Respiratory Questionnaire (SGRQ). After 4 months, patients were asked to select their subsequent therapy and indicate the reasons for their selection. RESULTS: A total of 78 patients completed the study. There were no significant differences in the improvements in FEV(1) or SGRQ scores between the therapies. Similar numbers of patients selected tiotropium (42.3%) and salmeterol plus fluticasone (57.7%). However, those who preferred one of the therapies demonstrated greater improvements in SGRQ scores with that therapy. One subgroup of patients (30.8%) showed greater improvements in dyspnoea and FEV(1) in response to tiotropium, and the other subgroup of patients (35.9%) showed greater improvements in dyspnoea and FEV(1) in response to salmeterol plus fluticasone. Some patients (14.1%) selected salmeterol plus fluticasone because of positive effects on sputum expectoration. CONCLUSIONS: The study was unblinded and the results need to be interpreted with caution. However, tiotropium and salmeterol plus fluticasone had similar overall effects on pulmonary function and SGRQ scores in patients with COPD. Responses to the two therapies were heterogeneous, and the patients who showed greater improvements in FEV(1) or SGRQ scores with one of the therapies preferred it for their subsequent treatment.

[Hypersensitivity pneumonitis induced by Hypsizigus marumoreus].

We present the case of a 53-year-old woman who was employed at a mushroom (Pleurotus eryngii and Hypsizigus marumoreus) cultivation factory for 15 years. She was admitted to our hospital because of fever and dry cough. Chest radiography and CT scanning revealed diffuse ground glass opacity and centrilobular nodules in both lung fields. Serum KL-6 was elevated. In the bronchoalveolar lavage fluid, the CD4/CD8 ratio was reduced, and the lymphocyte fraction was very high. Transbronchial lung biopsy specimens showed lymphocyte alveolitis. After admission, the patient's symptoms improved rapidly without medication. Although these findings are compatible with hypersensitivity pneumonitis, it was difficult to identify a causative antigen. Serum antibody against Trichosporon was positive. A lymphocyte stimulation test of the peripheral blood was positive against extracts of P. eryngii and H. marumoreus. Furthermore, precipitins against the extracts of H. marumoreus were detected by a double immunodiffusion test. Therefore, we decided to conduct a challenge test using H. marumoreus. As an inhalation provocation test with H. marumoreus conducted in a sickroom caused the same clinical symptoms and signs as experienced in the workplace, we diagnosed hypersensitivity pneumonitis caused by H. marumoreus. A provocation test, in which antigen exposure is limited using a closed space, such as a sickroom, was simple, safe and effective for determining the antigen causing hypersensitivity pneumonitis.

[Mucoid impaction of the bronchi caused by Schizophyllum commune which developed after discontinuation of itraconazole administration].

We report the case of a 75-year-old woman with mucoid impaction of the bronchi (MIB) due to Schizophyllum commune who improved with itraconazole (ITCZ) administration and relapsed after discontinuation of the drug. She improved again after readministration of ITCZ, and MIB has not recurred. This patient was not suffering from asthma and has been well without steroid administration. Reports of respiratory disorders due to S. commune have been increasing, and cases of allergic bronchopulmonary mycosis (ABPM), fungus ball, lung abscess, and pneumonia have been reported. Including this report, 12 cases of ABPM and MIB due to S. commune have been reported by Japanese authors. Treatment in these 12 cases included anti-fungal agent in 6, single steroid therapy in 3, combination therapy in 2, and bronchial toilet in 1 case. S. commune is not well recognized; however, one should suspect this fungus to be the causative pathogen when Aspergillus species are not detected or anti-Aspergillus antibody is negative.

[A case of Ehlers-Danlos syndrome suspected from pulmonary hematoma due to disruption of the lung].

A 20-year-old man was referred to our hospital due to hemoptysis. Chest CT showed a ground-glass opacity, suggesting pulmonary bleeding; however, a diagnosis was not obtained. At a follow-up examination after 2 months, Chest CT showed improvement of the ground-glass opacity, however a cavitary nodule had newly appeared. Four months later, another new nodule was found on chest X-ray film. Video-assisted thoracoscopic lung biopsy was performed for pathological diagnosis; disruption of the pleural, lung and blood vessels, and pulmonary hematoma were found. We thought of the fragility of the pleuro-pulmonary connective tissue and also thought of the probability of Ehlers-Danlos syndrome (EDS). A biochemical analysis of cultured dermal fibroblasts and molecular biological examination revealed decreased production of type III collagen in fibroblasts and COL3A1 mutation. We diagnosed this case as vascular EDS. EDS is one of the differential diagnoses in patients presenting hemoptysis and pulmonary hematoma due to disruption of the lung.

[Primary ciliary dyskinesia. A case report and comparison with 4 previous cases].

We encountered a 59-year-old man, whose chief complaints were sputum and dyspnea on effort. He had suffered from sinusitis since childhood, and chest computed tomography showed bronchiectasis. Electron microscopic examination of bronchial mucosa biopsied by bronchoscopy showed defect of the inner dynein arm in most of his cilia. We diagnosed primary ciliary dyskinesia. Seminal analysis showed no abnormalities, and the etiology of infertility remains unclear. The present case is the fifth case treated at our hospital. In this paper, we compared this case with 4 cases (2 cases of Kartagener's syndrome and 2 cases of primary ciliary dyskinesia without situs invertus) of our hospital, and findings of previous reports. Primary ciliary dyskinesia should be included in the differential diagnosis even in cases of mild bronchiectasis or normal mobility of sperm. Primary ciliary dyskinesia has more variety in radiological and clinical findings than has been recognized.

[Autopsy case of diffuse pleural thickening presenting respiratory impairment and benign asbestos pleurisy].

A 51-year-old man presented with back pain in 1997. He had a 30-year-history of occupational asbestos exposure. His chest CT showed bilateral pleural thickening and pleural effusion. The pleural effusion of the right thorax exhibited both elevated level of adenosine deaminase and increased numbers of lymphocytes. Antituberculous chemotherapy had no effect on the exudates. Progressive bilateral pleural thickening were found on chest CT, and pulmonary function tests showed severe restrictive ventilatory impairments since 1998. Thoracoscopic pleural biopsy was conducted in 2001 to exclude pleural malignant mesothelioma. No malignancy was found in pleural samples. After 3-year observation and excluding other causes, he was given a diagnosis of benign asbestos pleurisy. In 2005, fibrotic changes were found in both lower lung fields in chest CT. He suffered from respiratory failure with carbon dioxide retention, and died in 2006. The autopsy disclosed asbestos-related lung diseases. We suspected that diffuse pleural thickening could be a major cause of fatal respiratory impairment in this case.

[Long-term CT findings of X-linked agammaglobulinemia with bronchiectasis diagnosed in an adult].

We report a case of X-linked agammaglobulinemia who presented with bronchiectasis. The patient had suffered pneumonia about every five years since childhood until he presented to our hospital at age 34 years old. CT showed bronchiectasis predominantly in the right middle lobe, lingula, and lower lobes. Administration of antibiotics resulted in symptomatic relief. Episodes of recurrent pulmonary infection and bronchiectasis indicated congenital immunodeficiency disorder. Investigation of lymphocyte subsets and serum immunoglobulin values showed remarkable reduction of B cells, IgG 772 mg/dl, IgA 216 mg/dl, and IgM 29 mg/dl. Flow cytometric assessment combined with genetic analysis was performed, and the results showed decreased expression of monocyte Bruton's tyrosine kinase (BTK) and missense mutation of Btk gene. We diagnosed X-linked agammaglobulinemia. IgG remained above 600 mg/dl in this case, we have not administered immunoglobulin after discharge. He suffered from pneumonia in 2004 and 2006 and bronchiectasis has progressed. In this report, we present a case including CT findings over a period of 8 years.

[A case of the metastatic calcification of trachea, bronchus, and lung].

A 43-year-old man with chronic renal failure who had been treated by continuous ambulatory peritoneal dialysis (CAPD) was admitted to our hospital because of dry cough. Blood gas analysis showed hypoxemia and metabolic alkalosis. Laboratory data showed elevated levels of phosphorus, BNP, and KL-6. Lung function tests showed restrictive ventilatory failure and impairment of diffusing capacity. Chest CT revealed centrilobular ground-glass opacification in both lung fields, irregular reticular abnormality in left lung field, and calcification around the left shoulder joint. Bronchoscopy revealed a white protruding lesion in the trachea and bronchial membranous portion. Calcified metastasis in the bronchus and lung was confirmed by transbronchial and lung biopsy. Because of no improvement by administration of sevelamer, he started with hemodialysis once a week in addition to CAPD. Cough and bilateral ground-glass opacity were improved.

[A case of human T lymphotropic virus type I-related lung disease in which Legionella pneumonia led to the diagnosis].

A 46-year-old man whose parents were from Nagasaki had a 24-hour bath system in his house. He had had a cough for 26 years and dyspnea for 2 years. He consulted our hospital because of the increase of his cough and dyspnea. Chest X ray and CT showed abnormal shadows in both lung fields and Legionella pneumophila type 3 was detected by sputum cultivation. The condition which appeared this time responded to antibiotic medication. However, the abnormal shadows in both lung fields and the abnormalities in respiratory function remained. The remaining abnormal shadows in both lung fields were considered to be the cause of his dyspnea for 2 years and cough for 26 years. Atypical lymphocytes with a floriform nucleus were observed in peripheral blood. Gene analysis detected monoclonal human T lymphotropic virus type I (HTLV-I) provirus DNA. We diagnosed as smoldering type adult-T-cell-leukemia (ATL). Thoracoscopic lung biopsy revealed fibrotic thickening of the interstitial tissue accompanied by structural destruction. The pathological changes in both lung fields were diagnosed as HTLV-I related lung disease and infiltration of ATL. Known pathogens of lung infection accompanying ATL include viruses, acid fast organisms, and fungi. Legionella pneumonia happened to be the opportunity leading to the diagnosis of HTLV-I related lung disease is this case.

[A case of fulminant psittacosis with rhabdomyolysis].

We encountered a case of psittacosis accompanied with rhabdomyolysis in a 66-year-old woman admitted to our hospital because of myalgia, fever, diarrhea, and disorientation. A chest CT scan showed air-space consolidation and ground-glass attenuation in the left lung. Laboratory findings showed elevation of CPK, myoglobin, and aldolase, consistent with rhabdomyolysis. She recovered after administration of intravenous erythromycin and steroid pulse therapy. Psittacosis was diagnosed from the history of exposure to pigeons and from elevation of the IgG titer for Chlamydia psittaci. Rhabdomyolysis is associated with some kinds of viral or bacterial infections. But few case reports have been found in the case of psittacosis. The mechanism whereby rhabdomyolysis complicates psittacosis is unknown. We suggest that psittacosis should be added to the list for differential diagnosis, when rhabdomyolysis develops in a patient with pneumonia.

[Prognostic significance of serum uric acid in patients with chronic obstructive pulmonary disease receiving home oxygen therapy].

Home oxygen therapy (HOT) not only prolongs life expectancy but also improves quality of life. Serum uric acid (UA), the final product of purine catabolism, has been shown to be increased in the hypoxic state. To elucidate the prognostic significance of serum UA in patients with chronic obstructive pulmonary disease (COPD) receiving HOT, we assessed the ratio between the serum concentration of UA and creatinine (Cr) in 91 outpatients with COPD. During a mean follow-up period of 31 months, 24 patients died of acute exacerbation of COPD. The delta UA/Cr ratio was calculated as the percent changes in serum UA/Cr during HOT. delta UA/Cr was increased in non-survivors, but not in survivors, and was negatively correlated with the nadir of oxyhemoglobin saturation (r = -0.32, p < 0.01). Of the clinical and laboratory variables, only the delta UA/Cr ratio was found to be independently related to mortality by a multivariate Cox proportional-hazards analysis. The Kaplan-Meier survival curves divided into values below and above the median value (9.7%) of this ratio demonstrated that mortality was significantly higher among patients with high values than among those with low values (log-rank test: p < 0.05). We conclude that the delta UA/Cr ratio appears to be a reliable marker of prognosis, and may be useful for the long-term follow-up of outpatients with COPD receiving HOT.

[A case of chronic pigeon breeder's disease accompanied with progressive pulmonary cysts and recurrent pneumothorax].

We followed up a case of chronic pigeon breeder's disease with progressive pulmonary cysts and recurrent pneumothorax. The patient was a 48-year-old man who started to breed pigeons 14 years ago. He was diagnosed 7 years ago as having hypersensitivity pneumonitis due to pigeons, but he refused to stay away from the birds until he experienced episodes of dyspnea on exertion 3 years ago. A chest CT scan showed multiple cysts and peribronchial fibrosis in both lungs. The pathological findings of video-assisted-tracheoscopic surgery showed a centribronchial granuloma with lymphocyte infiltrations. Tests for antibodies against pigeon extracts of droppings were positive. Even after steroid treatment and avoidance of pigeons 3 years ago, there have been progressive cysts and fibrosis in the lungs. Progressive pulmonary cysts with recurrent pneumothorax is a rare manifestation as a chronic pigeon breeder's disease.

[A case of combined hypersensitivity pneumonitis and bronchial asthma due to isocyanate (MDI)].

A 29-year-old man was admitted to our hospital complaining of cough, wheezing, dyspnea, and fever one month after handling paint spray containing isocyanate (MDI). Chest HRCT findings showed diffuse ground-glass attenuation in both lung fields. A pulmonary function test revealed restrictive impairment, and the reversibility test was positive. His symptoms, HRCT findings, and pulmonary dysfunction were improved only after the cessation of isocyanate administration. BALF showed lymphocytosis, and the pathological findings of the TBLB specimen revealed cellular alveolitis, but no Masson bodies or epitheloid cell granuloma. As a result of environmental provocation, fever, hypoxia, and reduced peak expiratory flow developed, and the environmental provocation test was positive. The specific antibodies against MDI and TDI were positive in both serum and BALF, and the lymphocyte stimulation test against MDI was positive in peripheral blood. Combined hypersensitivity pneumonitis and bronchial asthma due to isocyanate were therefore diagnosed. Pulmonary dysfunctions due to isocyanate are known to include bronchitis, bronchial asthma, and hypersensitivity pneumonitis. However, case reports of combined hypersensitivity pneumonitis and bronchial asthma due to isocyanate are rare.

[Usefulness of Streptococcus pneumoniae urinary antigen detection kit and the duration and intensity of reactivity with urinary antigen in patients with pneumonia].

We evaluated a rapid urinary antigen detection kit, Binax Now Streptococcus pneumoniae (Binax Inc., USA), which detects S. pneumoniae antigen in urine by immunochromatographic membrane assay, in 379 patients with presumptive pneumonia (total: 454 urine samples). S. pneumoniae antigen was detected in 64 (34%) of 188 patients. In all 64, pneumonia was diagnosed clinically, and there were 11 intense reactivity cases, 27 intermediate cases, and 26 weak cases. We found only two patients with positive sputum cultures for S. pneumoniae among 26 patients with weak reactivity to urinary antigen. The weak urinary antigen reactivity seems to include a false-positive result for S. pneumoniae pneumonia. There were five patients with negative results in whom S. pneumoniae was isolated (false-negative). We took intense and intermediate reactivity to be positive in order to diagnose pneumococcal pneumonia, and the kit showed a sensitivity of 72% and a specificity of 94% in 379 patients. The urinary antigen kit allowed us to diagnose 80% more patients with pneumococcal pneumonia than the use of conventional bacteriological diagnosis alone. There was no significant difference in the initial clinical characteristics, or in the severity of pneumonia among the three groups, according to the color intensity reached using the kit--weak, intermediate, and intense for the reactivity of urinary antigen. The duration of reactivity with S. pneumoniae urinary antigen did not correlate with the clinical characteristics or the severity of pneumonia. We concluded that S. pneumoniae urinary antigen detection kit is a useful adjunct to culturing for determining the etiology of pneumonia.