[Two cases of Mycobacterium avium complex pleuritis].

We encountered 2 cases of Mycobacterium avium complex pleuritis. One case was a 68-year-old woman suffering from rheumatoid arthritis, treated with steroids. She presented to our hospital with dyspnea, which improved after a chest tube insertion and the administration of rifampicin, ethambutol, clarithromycin, and streptomycin. In the other case, a 71-year-old man suffering from nephritic syndrome and who had been treated with steroids presented to our hospital with low-grade fever and dyspnea. He improved after therapeutic thoracentesis and clarithromycin administration. Only 6 cases of Mycobacterium avium complex pleuritis have been reported; because it is rare, the characteristics and prognosis of the disease remain unknown. We report 2 cases and review 6 previous reports of Mycobacterium avium complex pleuritis.

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.

[Bilateral primary racemose hemangioma of the bronchial artery detected by nodular stenosis of the bronchial lumen in a patient with chronic cough and a review of the Japanese literature].

A 55-year-old man was admitted to our hospital with chronic cough. Although his chest X-ray was normal, chest computed tomography revealed a nodular lesion that was causing beaded stenosis of the lumen of the upper lobe bronchus. Nodules approximately 10 mm in size were seen in the mediastinum. We could not deny the possibility of vascular disease, and enhanced chest computed tomography was performed, which showed these abnormalities to be caused by a dilated and convoluted bronchial artery. Because no pulmonary diseases that would lead to secondary vascular dilatation were evident, a diagnosis of primary racemose hemangioma of the bronchial artery was made. We performed bronchial artery embolization to prevent potential fatal bleeding from vascular rupture. The patient has been followed regularly as an outpatient, and recanalization has not been detected. Primary racemose hemangioma of the bronchial artery is a rare entity; it is also rare for this entity to be detected by findings of a nodular protrusion in the bronchial wall or in a patient presenting with a cough. Here, we report this case and review previous reports.

[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.

[Severe complications and their outcomes in 65 patients with Legionella pneumonia].

The aim of the current study was to investigate the lethal complications of Legionella pneumonia. Severe complications and their outcomes in 65 patients with Legionella pneumonia were studied. All patients who eventually had a fatal outcome or who had severe complications received antimicrobial agents active against Legionella on the admission day. Many patients in the severe complication category had multiple severe complications. Six deaths occurred (mortality rate 9.2%), 4 of which were due to septic shock/multiple organ dysfunction syndrome (MODS) (2 patients) or interstitial pneumonia/pulmonary fibrosis after Legionella pneumonia (2 patients), whereas the other 2 deaths were due to causes unrelated to Legionella pneumonia. Mortality rates for each severe complication were as follows: acute respiratory distress syndrome 27.3% (3 of 11); renal failure 33.3% (2 of 6); disseminated intravascular coagulation 33.3% (2 of 6); severe sepsis 0% (0 of 1); septic shock/MODS 66.7% (2 of 3); interstitial pneumonia/pulmonary fibrosis 50% (2 of 4). Despite prompt diagnosis and appropriate treatment with antimicrobial agents active against Legionella, the lethal complications of Legionella pneumonia are septic shock/MODS and interstitial pneumonia/pulmonary fibrosis.

[Nemaline myopathy detected with respiratory failure and right ventricular heart failure].

We describe the case of a 40-year-old woman who was admitted for dyspnea and pitting edema of the lower extremities. Severe type II respiratory failure and right ventricular heart failure were present. Non-invasive positive pressure ventilation (NIPPV) improved the symptoms and blood gas values. Since the results of respiratory function tests and computed tomography indicated neuromuscular disease, muscle biopsy was performed and nemaline myopathy was diagnosed. NIPPV was necessary due to severe hypoxia and hypercapnia caused by severe hypoventilation during sleep; however, daytime NIPPV was stopped within a few days, and the patient was discharged with instructions to continue NIPPV at night only. Since discharge, she has been followed-up on an outpatient basis for 8 years. Adult-onset nemaline myopathy with respiratory failure and right ventricular heart failure as presenting features is rare, and NIPPV can be useful in such cases.

[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.

[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.

[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.

Effect of early versus late intervention with inhaled corticosteroids on airway wall thickness in patients with asthma.

BACKGROUND AND OBJECTIVE: The aim of this study was to determine whether early versus late initiation of long-term inhaled corticosteroid (ICS) therapy decreases airway wall thickness in patients with asthma. METHODS: One hundred and eighty-one patients with asthma not previously treated with ICS were given inhaled budesonide for 1 year. These patients were divided into five groups according to the duration of their asthma symptoms, which ranged from less than 1 year to more than 10 years. High-resolution CT images and post-bronchodilator FEV1 were examined before and 1 year after treatment. RESULTS: Before treatment, airway wall thickness was increased relative to the duration of asthma. Disease severity improved with ICS treatment even in patients who had suffered asthma symptoms for more than 10 years. Post ICS treatment, airway wall thickness decreased in patients with a duration of symptoms less than 3 years, and a minor response was seen in patients with a duration of symptoms from 3 to 5 years. However, there was no change in airway wall thickness in patients who had suffered asthma for more than 5 years. Post-bronchodilator FEV1 improved only in patients who had suffered asthma for less than 3 years. CONCLUSIONS: ICS therapy may improve asthma control in all asthma patients despite the disease duration, but early ICS treatment may be critical to reverse airway wall thickening associated with asthma.

[A case of rheumatoid arthritis complicated with methotrexate-induced pneumonitis and pneumocystis pneumonia].

A 62-year-old woman with rheumatoid arthritis was given 4 mg/body methotrexate (MTX) every week and 5 mg prednisolone every day. She developed a severe cough starting in the evening after starting taking MTX and after a fever of 38 degrees and dyspnea appeared the patient was hospitalized. On admission, chest CT findings showed diffuse ground glass attenuation. Pathological findings of specimens obtained by transbronchial lung biopsy showed alveolitis with epithelioid cell granuloma. As a section of the specimen did not show cyst staining by Grocott stain, MTX-induced pneumonitis was diagnosed. The same day, methylprednisolone pulse therapy was started and trimethoprim-sulfamethoxazole (TMP-SMX) was given simultaneously, while MTX was discontinued. On hospital day 3, subsequent data showed a high serum level of beta-D glucan and a positive PCR result for Pneumocystis jiroveci in bronchoalveolar lavage fluid (BALF). Additional section of the specimen showed eosinophilic foamy areas on HE staining and cysts measuring 8 microm, consistent with the Pneumocystis jiroveci lesions by Grocott stain. We present a case of rheumatoid arthritis complicated by methotrexate-induced pneumonitis in which pneumocystis pneumonia was demonstrated by clinical and pathological findings.

[Clinical features of mixed infections in patients with Streptococcus pneumoniae pneumonia].

We retrospectively analyzed the severity, the mortality and the initial antimicrobial therapy in 195 patients with Streptococcus pneumoniae pneumonia (SPP). Of these, 59 (30.3%) patients had mixed pneumonia. In patients with mixed SPP, the three most frequent pathogens were influenza virus (27 patients), Haemophilus infuluenzae (14 patients), and Mycoplasma pneumoniae (8 patients). Of these, 21 (35.5%) patients were classified as severe or very severe according to the Japanese Respiratory Society diagnostic criteria among 59 patients of mixed SPP. Severe and very severe pneumonia was significantly associated with mixed infections (P = 0.018). The initial antimicrobial therapy was classified as beta-Lactam alone (113 patients), combination therapy including a beta-Lactam (72 patients), and a fluoroquinolone alone (10 patients). If we limit out study to mild-moderate pneumonia, initial combination therapy was significantly effective in patients with mixed SPP. Even in pneumonia caused by Streptococcus pneumoniae, further efforts to identify etiology are necessary.

[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.

[Case of hot tub lung with confirmation of exacerbation following jet bath use].

A 49-year-old woman presented with exertional dyspnea. Chest CT revealed patchy areas of ground-glass attenuation and ill-defined centrilobular nodules scattered in both lungs. Bronchoalveolar lavage (BAL) fluid showed lymphocytosis. Transbronchial lung biopsy revealed bronchiolocentric alveolitis and well-formed non-necrotizing granulomas were present. She had used a jet bath before the onset of symptoms and mycobacterial culture revealed the presence of Mycobacterium avium complex (MAC) in sputum sample, BAL samples and jet bath water. Restriction fragment length polymorphism (RFLP) analysis revealed that the isolated MAC were essentially clonal. She had used the jet bath for the inhalation provocation study, and after the challenge she complained of dyspnea and have body temperature increased. We diagnosed hot tub lung due to Mycobacterium avium complex. Because avoidance of the jet bath caused improvement of her symptoms and reduced her fever and PaO2 increased by 10 Torr but did not improve the CT findings, antimycobacterial drugs were prescribed. The patient recovered fully. This case proves that the cause of hot tub lung is the use of jet bath through the inhalation provocation study.

[Case of pneumonia caused by beta-lactamase-producing and amoxicillin/clavulanate resistant strains of H. influenzae].

An 80-year-old woman presenting with fever and cough was given a diagnosis of community-acquired pneumonia. She was hospitalized and treated with ampicillin/sulbactam (ABPC/SBT) and clarithromycin (CAM). Gram stain images and sputum culture results led us to believe that the causative agent was Haemophilus influenzae. Drug sensitivity testing indicated that the H. influenzae was a beta-lactamase-positive, ABPC-resistant (BLPAR) strain. Treatment with ABPC/SBT was not clinically effective. We considered the possibility of beta-lactamase-positive amoxicillin/clavulanate-resistant (BLPACR) strains. Further testing revealed that the MIC of ABPC was 128 microg/ml, that of SBT/ABPC was 8 microg/ml, and that of AMPC/CVA was 4 microg/ml. Furthermore, genetic analysis indicated the H. influenzae to be a BLPACR-I strain. The poor clinical course eventually led to a diagnosis of BLPACR. When beta-lactamase-producing H. influenzae is cultured, the possibility of a BLPACR strain resistant to ABPC/SBT and AMPC/CVA must be considered.

[Lung cancer associated with rheumatoid arthritis and usual interstitial pneumonia].

UNLABELLED: To assess the effects of usual interstitial pneumonia (UIP) and smoking in rheumatoid arthritis (RA) patients regarding lung cancer risk, we studied 86 RA patients (14 patients with lung cancer, 58 patients with UIP (RA/ UIP), and 14 patients with both). Among the 28 RA patients with lung cancer, 14 patients (50%) had UIP. Compared to the lung cancer patients without UIP, the proportion of smokers (92.6 vs 50%, p = 0.0328) and total pack-years of smoking (57.1 +/- 37.1 vs 19.5 +/- 21.9, p = 0.003) were significantly higher in lung cancer patients with UIP. There was no significant difference in age, sex, and histological type and location of lung cancer between these two groups. Among the 72 RA/UIP patients, 14 patients (19.4%) had lung cancer. Compared to RA/UIP-only patients, the proportion of smokers (92.6 vs 46.6%, p = 0.002) and total pack-years of smoking (57.1 +/- 37.1 vs 25.4 37.4, p = 0.006) were significantly higher in RA/UIP patients with lung cancer. There was no significant difference in age, sex, and the duration of RA between these two groups. The odds ratio of smoking and total pack-years of smoking over 20 for the development of lung cancer in RA/UIP patients were 14.93 and 21.27, and the differences were statistically significant (p = 0.002 and p < 0.001, respectively). CONCLUSION: 50% of RA patients with lung cancer had RA/UIP and 19.4% of RA/UIP patients had lung cancer. These findings suggest that RA/UIP may be associated with increased risk of development of lung cancer in RA patients. In an RA cohort study to assess the development of lung cancer, RA patients should be divided into two groups, one with RA/UIP and another without RA/UIP. Smokers with RA/UIP should be recommended to cease smoking.

[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.

Pulmonary Schizophyllum commune infection developing mucoid impaction of the bronchi.

A 54-year-old woman was admitted for cough, sputum, and an abnormal chest X-ray shadow. Bronchoscopy showed mucoid impaction of the bronchi (MIB). Histopathologic evidence of mucous plugs was consistent with one component of allergic bronchopulmonary mycosis. Schizophyllum commune (S. commune) was identified. Two attempts at removal of the mucous plugs were unsuccessful. Itraconazole was then administered, and the mucous plugs disappeared. There are few reports of MIB due to S. commune; we herein report a case of MIB due to S. commune infection.

[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.

[Pneumocystis carinii pneumonia in a patient with hyper-IgE syndrome].

A 15-year-old girl with abnormal findings detected on a medical check-up chest x-ray film was admitted to our center. High-resolution computed tomography, performed upon hospitalization, demonstrated panlobular nodular darkening in left lung fields, and an expanding, blended, map-like darkening near the pleura. Since a Grocott stain-positive cyst was confirmed histopathologically by transbronchial lung biopsy, the patient was given a diagnosis of Pneumocystis carinii pneumonia. Drug therapy was initiated with sulfamethoxaxole trimethoprim (Baktar), and on the 58th day, chest CT confirmed that the darkening observed at admission had virtually disappeared. Underlying diseases, such as AIDS, malignant lymphoma and secondary immunodeficiency caused by immunosuppressive agents or adrenocorticosteroids, were excluded as the cause of P. carinii pneumonia based on clinical/laboratory findings. Under the suspicion of the possibility of primary immunodeficiency, various immunological competence tests were performed. However, no abnormal findings indicating cell-mediated immunity, humoral immunity, complement immune function, neutrophil phagocytic capacity, or bactericidal capacity were recognized. Since significant increase of serum IgE suggested hyper-IgE syndrome, IgE antibody specific to Staphylococcal enterotoxin A and B, and the exotoxins of Staphylococcus aureus were measured with positive results. Since all three diagnostic criteria for hyper-IgE syndrome (i.e., high serum IgE values, positive IgE antibody specific to Staphylococcal enterotoxin and recurrent infection) were fulfilled, hyper-IgE syndrome was diagnosed. This is a rare case of hyper-IgE syndrome as a result of P. carinii pneumonia.