[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.

Pleuropulmonary pathology of vascular Ehlers-Danlos syndrome: spontaneous laceration, haematoma and fibrous nodules.

AIMS: The aim was to clarify the pleuropulmonary pathological findings of vascular Ehlers-Danlos syndrome (vEDS). METHODS AND RESULTS: Nine patients with confirmed vEDS by means of cell culture and/or molecular biological studies who had undergone surgical lung biopsy (SLB), lobectomy or autopsy were studied. Six patients were male and three were female with a mean age of 23.2 years. Histological features were as follows: (i) the main pulmonary lesions related to fragility and spontaneous laceration, these being haematomas in seven, acute haemorrhage in nine, fibrous nodule in eight, with ossification or bone marrow formation in six; vascular disruption in five; intraluminal haemosiderosis in nine; interstitial haemosiderosis in seven, with iron deposition in the alveolar wall and/or vessel wall in five and foreign body reaction in two; emphysematous changes in eight; and bleb formation in two; (ii) secondary iatrogenic pleuropulmonary injuries during SLB or lobectomy comprised pleural laceration in seven of 10 and lung laceration in eight of 10 specimens. CONCLUSIONS: Spontaneous laceration of lung tissue is an essential feature and is followed by haematoma and possible fibrous nodule formation.

[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.

[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.

[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.

[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 miliary tuberculosis showing acute respiratory failure during pregnancy].

A 36-year-old Philippine woman had had fever and general fatigue from September, 2006 (11th week of pregnancy). She was admitted with high fever, general fatigue and dyspnea on October 16, 2006 (13th week of pregnancy). A chest radiograph on admission showed bilateral miliary shadows and ground glass shadows. She already had severe hypoxia on admission. As acid-fast bacilli were positive in urine (Gaffky 8) and sputum (Gaffky 1), we diagnosed as miliary tuberculosis and pulmonary tuberculosis complicated with acute respiratory distress syndrome (ARDS). We treated her with antituberculosis chemotherapy, corticosteroid, sivelestat sodium hydrate, direct hemoperfusion using a polymyxin B immobilized column, and mechanical ventilation, but she died due to respiratory failure. We emphasize that in this case pregnancy has the risk of to causing disease progression of miliary tuberculosis and we should treat immediately and intensively for miliary tuberculosis complicated with ARDS.

[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.

Etiology and factors contributing to the severity and mortality of community-acquired pneumonia.

OBJECTIVE: Community-acquired pneumonia (CAP) remains a major cause of death. No studies have reported the use of rapid influenza diagnostic tests (RIDT) for the etiological diagnosis, and the factors contributing to severity and mortality have not yet been fully investigated. The aim of this study was to review the etiologies of CAP using RIDT and to identify risk factors related to the severity and mortality of the disease. METHODS: This retrospective study assessed these factors in hospitalized patients, with special emphasis on microbial etiology. RESULTS: A total of 1,032 patients aged 63.9±18.3 years were studied, 66.2% of whom were men. Microbial identification was obtained in 57.0% of the cases. The most frequent causative microbial agents were Streptococcus pneumoniae, Mycoplasma pneumoniae and the influenza virus, and the second most frequent pathogens in the patients with severe CAP and the non-survivors were S. pneumoniae and the influenza virus. Age (≥65 years), chronic obstructive pulmonary disease, congestive heart failure, diabetes mellitus, dementia and Legionella spp. infection and polymicrobial infection were each found to be independent factors related to severity in the multivariate analysis, whereas "unidentified pathogen" was found to be an independent factor for non-severe CAP. Age (≥65 years), chronic pulmonary aspergillosis, post-lung cancer surgery and severe CAP were found to be independent factors for non-survival according to a multivariate analysis. CONCLUSION: In addition to S. pneumoniae, the influenza virus was a frequent cause of CAP overall and a frequent causative pathogen in both severe cases of CAP and non-survivors. Legionella spp. infection and polymicrobial infection were found to be an independent factor for the severity of CAP along with advanced age and certain comorbidities. An advanced age, certain respiratory comorbidities and severe CAP were found to be important independent factors for the mortality of CAP.

Pulmonary capillaritis in Wegener's granulomatosis detected via transbronchial lung biopsy.

A 73-year-old woman presented to our hospital with scleritis, cough, and hemoptysis. Chest computed tomography showed a mass lesion, multiple ground-glass opacities, and nodules. Bronchoscopy showed a skip lesion of mucosal erosion with white necrotic substance; however, a biopsy specimen of the erosion yielded no characteristic findings. Transbronchial lung biopsy showed alveolar hemorrhage and capillaritis. Proteinase-3 antineutrophil cytoplasmic antibody was elevated to 62.5 U/mL, and she was diagnosed as having Wegener's granulomatosis. Steroid therapy combined with cyclophosphamide was started; however, hemoptysis was not resolved until plasmapheresis therapy was added. Her general condition initially improved, but respiratory insufficiency worsened with fever on the 30th hospital day. The patient subsequently died on the 41st hospital day, and cytomegalovirus infection was suggested as a possible cause of death.

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.

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.

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.