[Legionella pneumonia in a patient with positive culture results of Legionella pneumophila serotype 1 from bronchoalveolar lavage fluid but negative urinary antigen test results].

A 39-year-old man presented to a local physician complaining of fever, headache without cough or sputum. Abnormal shadows were found on chest X-ray films. Pneumonia was diagnosed and he was referred to our hospital, where chest computed tomography showed bilateral, multifocal, and patchy ground-glass opacities. Neither the urinary antigen test for Streptococcus pneumoniae nor that for Legionella was positive. Because he had visited a public spa several days before developing the fever and headache, and because hypophosphatemia was identified, we then suspected Legionella pneumonia. Bronchoalveolar lavage fluid yielded positive culture of Legionella pneumophila serotype 1. The Legionella urinary antigen is known to detect Legionella pneumophila serotype 1 infection; however, sensitivity and specificity of the test are 60-95% and > 99%, but care should be taken when urinary antigen test results are negative in suspected cases of Legionella pneumophila serotype 1 pneumonia. In the present case, bronchoalveolar lavage fluid was a useful method for diagnosing Legionella pneumonia.

[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.

[A case of organizing pneumonia which recurred 51 months after radiotherapy following breast-conserving surgery].

A 54-year-old woman who had undergone breast-conserving surgery followed by irradiation presented to a local physician after developing a cough and low-grade fever 9 months after radiation therapy. She was given a diagnosis of pneumonia and antibiotics were administered, but since she did not improve, she was transferred to our hospital. We performed lung biopsy via thoracoscopy and diagnosed organizing pneumonia based on pathological findings. Steroid administration based on her chest X-ray findings, improved her condition. After discharge, her steroid dose was tapered on an outpatient basis, but she suffered relapse of her organizing pneumonia three times. The prednisolone dose at relapse was 10 mg/day at 20 months, 7.5 mg/day at 36 months, and 7 mg/day at 51 months after radiation therapy, respectively. Organizing pneumonia that develops after irradiation following breast-conserving surgery has been known to occasionally relapse, and to the best of our knowledge the final relapse reported here, 51 months after radiation therapy is the longest relapse reported.

[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.

[Case of bird-related hypersensitivity pneumonitis in a farmer].

A 48-year-old female farmer presented to our hospital with shortness of breath and cough. Her partial pressure of arterial oxygen (PaO2) level on room air was 58.4Torr, and chest computed tomography (CT) showed centrilobular ground-glass micronodules. She was admitted for further evaluation. After admission, symptoms, PaO2, and radiological findings improved without any treatment; however, her symptoms recurred when she returned to work. Exposure to her house did not elicit any symptomatic response. We initially suspected farmer's lung, but inhalation provocation tests with hay showed no significant change in symptoms, laboratory data or radiological findings. Antibody tests for Thermoactinomyces vulgaris or Saccharopolyspora rectivirgula were negative. Because many pigeons were found roosting on her farm, we suspected bird-related hypersensitivity pneumonitis. Precipitating antibodies testing against pigeon serum and IgG and IgA antibodies testing against pigeon dropping extracts were all positive, and bird-related hypersensitivity pneumonitis was diagnosed. Because typically, many birds roost on farms, not only farmer's lung, but also bird-related hypersensitivity pneumonitis, should be included in a differential diagnosis when a farmer clinically suspected to have hypersensitivity pneumonitis is encountered.

[A case of scleritis as the initial clinical manifestation of limited Wegener's granulomatosis].

A 63-year-old man who was transferred to our hospital had noticed ocular hyperemia, and 2 months later headache, dry cough, nose bleeds and fever. Chest X-ray film showed bilateral infiltrating shadows. He was given a diagnosis of pneumonia at the previous hospital, where antibiotics were administrated with no effect. High-resolution chest computed tomography (HRCT) on admission revealed consolidations distributed predominantly in the bilateral lower lobes and multiple nodules. Consolidations were also mainly distributed around bronchovascular bundles and at subpleural areas. We suspected Wegener's granulomatosis because of his eye lesion and HRCT findings. Ophthalmologic examination after admission revealed bilateral scleritis. The proteinase 3-antineutrophil cytoplasmic antibody level was 51.4 IU/ml. Thoracoscopic lung biopsy (left S8) showed eccentric granulomatous necrotizing vasculitis, which was consistent with Wegener's granulomatosis. We finally diagnosed this case as limited type Wegener's granulomatosis without renal involvement. We administrated both 60 mg/day prednisolone and 100mg/day cyclophosphamide for initial treatment. After this treatment, he remarkably improved. Scleritis was the initial clinical presentation in this case of Wegener's granulomatosis.

[A case of empyema due to Capnocytophaga sp. and Actinomyces israelii].

A 54-year-old man was admitted to our hospital for investigation of cough, sputum production, and fever of 1 month's duration. His diabetes mellitus was poorly controlled, and his hemoglobin HbA1c value was elevated at 10.9%. Chest X-ray film and computed tomography scan showed bilateral but predominantly right-sided pleural effusion. Aspiration of the pleural fluid from the right-side showed frank pus, and empyema was diagnosed. Capnocytophaga sp. and Actinomyces israelii were isolated in the pleural effusion and were regarded as the pathogens causing the empyema. Klebsiella pneumoniae was isolated in his sputum, and it may also have been a possible pathogen. The patient improved with administration of antibiotics (6 g/day ampicillin/sulbactam, 3 g/day ceftazidime hydrate and 1200 mg/day clindamycin) and chest tube drainage. He was discharged and regularly followed on an outpatient basis. We report this rare case of Capnocytophaga sp. and Actinomyces israelii as the pathogenic causes of empyema.

[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.

[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.

Ehlers-Danlos syndrome with recurrent spontaneous pneumothoraces and cavitary lesion on chest X-ray as the initial complications.

A 17-year-old-man developed left-sided pneumothorax in 1995. Chest computed tomography (CT) showed a thick-walled cavity in the left lower lobe. Video-assisted thoracic surgery was performed, and pathologic findings of the resected lung showed a cavity, organizing hematoma, and a fibrous nodule. Fragility of connective tissue was suspected, and biochemical and molecular analysis showed reduction of type III collagen production and point mutation of the COL3A1 gene. The patient was diagnosed as having vascular-type Ehlers-Danlos syndrome (EDS). From 2002, the patient developed hemoptysis and bloody sputum once a year. Chest CT detected several nodules and cavities, which were regarded as hematomas with or without excretion. Several vascular changes including aneurysmal formations have been found since 2002, and an aneurysm of the left ulnar artery was resected. The patient continues to be followed regularly on an outpatient basis. We report a rare case of vascular-type EDS who developed pulmonary symptoms as an initial complication.

[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.

[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.

[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.

Development of sarcoidosis during etanercept therapy.

This report describes a 65-year-old woman who developed granulomatous lesions consistent with sarcoidosis during etanercept therapy for rheumatoid arthritis. Hilar and mediastinal lymphadenopathy and multiple nodules in both lung fields developed 21 months after administration of etanercept. Noncaseating epithelioid cell granulomas consistent with sarcoidosis were detected in a lung biopsy specimen and in the parietal pleura obtained via thoracotomy. Diseases showing similar histologic changes were excluded, and a diagnosis of sarcoidosis was made. Etanercept was discontinued, which resulted in symptomatic relief, improvement of oxygenation and radiologic findings. There is substantial evidence of tumor necrosis factor-alpha involvement in the induction and maintenance of granuloma formation; however, we should keep in mind that granulomatous disease, such as sarcoidosis, can develop during treatment with a tumor necrosis factor-alpha blocking agent, such as etanercept.

[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.

Desquamative interstitial pneumonia with a remarkable increase in the number of BAL eosinophils.

A 57-year old man with desquamative interstitial pneumonia (DIP) showed a marked increase in eosinophils in the bronchoalveolar lavage (BAL) fluid. The patient was referred to our hospital for abnormal shadows on his chest X-ray with no symptoms in May 2007. Computed tomography (CT) showed patchy, peripheral predominate ground-glass opacity. The BAL fluid revealed an increase of the total number of cells, including markedly elevated levels of eosinophils (62.1%), in contrast with only a slight increase of peripheral blood eosinophils, or minimal eosinophils in the alveolar spaces and interstitium of the thoracoscopic lung biopsy specimen. Since the specimens showed findings compatible with a DIP pattern, we diagnosed the patient with DIP. Although it is a rare entity, we should therefore consider DIP in the differential diagnosis when we encounter patients with a marked increase in the number of BAL eosinophils.

[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.

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.