A case of adult onset idiopathic pulmonary hemosiderosis markedly improved by steroid therapy]

A case of adult onset idiopathic pulmonary haemosiderosis (IPH) was reported. A 53-year-old man was admitted to our hospital because of repeated bloody sputum on June 2, 2006. Chest radiograph on admission disclosed diffuse infiltrative shadows in both lung fields, and one month later these shadows became more marked. The chest CT on July 5, 2006 showed patchy areas of ground-glass opacity and consolidation, exhibiting a distinctly peripheral distribution. Bronchoscopic findings revealed oozing bleeding from the orifice of B5 in the right lung and B9 in the left lung. We employed video-assisted thoracoscopic surgery for lung biopsy and he as primary IPH was diagnosed clinicopathologically. His symptoms and radiographic findings were markedly improved after steroid therapy, followed by no signs of recurrence. It may be important to establish a definitive diagnosis early, even in IPH, using VATS, for further effective therapy.     

Double filtration plasmapheresis in case of Goodpasture's syndrome

This article describes a case of Goodpasture's syndrome controlled by double filtration plasmapheresis (DFPP) combined with steroid and immunosuppressant therapy. A 48-year-old male, clerk, complaining of fever, dry cough and macroscopic hematuria, was admitted to our hospital. Microscopic hematuria was first pointed out at age 40 on an annual check up. His laboratory data on admission revealed severe anemia, azothemia, macroscopic hematuria and proteinuria. His chest radiograph and CT revealed diffuse nodular densities in bilateral lung fields. Specimens obtained by transbronchial lung biopsy and open renal biopsy revealed linear deposition of IgG by direct immunofluorescent antibody methods. Circulating antiglomerular basement membrane antibody level determined with radioimmunoassay was 1.8% on admission, but one week later it elevated to 5.6% with progression of dyspnea, hypoxemia, and renal failure. Steroid pulse therapy and a total of 6 double filtration plasmaphereses were performed in the first month. Subsequently hypoxemia and dyspnea disappeared, and the chest radiograph of the 40th hospital day showed no abnormal shadows. Two months later recurrence of pulmonary hemorrhage was noticed. Immunosuppressant administration (Cyclophosphamide 100 mg/day) and a total of 10 DFPP procedures were performed with success. By DFPP, circulating anti-GBM antibody fell rapidly to within normal ranges, and anti-GBM antibody level elevated in removed plasma. We think DFPP is effective to remove circulating anti-GBM antibody in Goodpasture's syndrome.     

A case of eosinophilic lung disease presenting asthma-like symptoms and centrilobular shadows in both lung fields]

This case, in a 23-year-old man presenting with cough, sputum, dyspnea on effort and wheezing, had been diagnosed as bronchial asthma at another hospital. Because inhaled steroid and theophylline were far from effective, he was admitted to our hospital for further evaluation. A blood test revealed marked eosinophilia. Chest radiography showed diffuse, small nodular shadows in both lung fields, and a chest CT scan demonstrated diffuse centrilobular nodules and thickening of the bronchi and bronchioles. A spirometric test showed obstructive and restrictive ventilatory impairment, but the depressed forced vital capacity failed to show improvement in response to bronchodilator inhalation, discouraging a diagnosis of asthma. Eosinophilic lung disease with prominent eosinophilic bronchiolitis was diagnosed on the basis of BAL eosinophilia and thoracoscopic lung biopsy findings. The symptoms and blood eosinophilia were responsive to administration of oral prednisolone (30 mg daily); radiographic and CT findings also showed improvement. This case showed a marked similarity to the recently reported "eosinophilic bronchiolitis", and was probably not a type of bronchial asthma.     

Eosinophilic pulmonary syndrome as a manifestation of GVHD following hematopoietic stem cell transplantation in three patients

Eosinophilic pulmonary syndrome is an uncommon problem in SCT recipients that can mimic an infectious process. We report the occurrence of eosinophilic pulmonary syndrome in three patients following allogeneic hematopoietic stem cell transplantation (HSCT), and postulate that this entity is part of the clinicopathologic spectrum of pulmonary GVHD. In all three cases, active chronic GVHD of the skin preceded or coincided with the development of pulmonary involvement. Other common features included peripheral blood eosinophilia, diffuse bilateral pulmonary infiltrates and lung biopsies showing pronounced infiltrates of eosinophils involving the small bronchioles. All patients responded promptly to systemic steroid therapy, with improvement of their pulmonary symptoms and the resolution of peripheral blood eosinophilia. Clinicians should be aware that eosinophilic pulmonary syndrome can occur following HSCT, may be associated with other manifestations of chronic GVHD, and generally responds well to corticosteroid therapy.     

Bronchiolo-alveolar cell carcinoma arising after active pulmonary tuberculosis' report of two cases]

We report on two patients diagnosed as having active pulmonary tuberculosis who later developed lung cancer. In both cases, the lung cancer was detected during the treatment of pulmonary tuberculosis. Both patients were initially considered to be experiencing exacerbation of pulmonary tuberculosis. Case 1 was seen in a 74-year-old man. His chest roentgenogram revealed microscopic cavitary lesions with infiltration into both lung fields. His sputum tested positive for acid-fast bacilli. Although he was treated with isoniazid (INH), rifampicin (RFP), ethambutol (EB) and pyrazinamide (PZA), his general condition deteriorated, and the infiltrative shadows in the lung fields had expanded on subsequent chest radiography. Transbronchial lung biopsy (TBLB) yielded findings compatible with a diagnosis of bronchiolo-alveolar cell carcinoma. Case 2 occurred in a 52-year-old man. His chest radiograph revealed cavitary lesions with infiltration into both lung fields. His sputum also tested positive for acidfast bacilli. Despite medication with INH, RFP, EB and PZA, the infiltrative shadow in his chest radiograph increased in size. Bronchiolo-alveolar cell carcinoma was confirmed after examination of the sputum cytology. Case 1 was diagnosed as lung cancer 10 months after being admission to the hospital, and Case 2, seven months after hospitalization. Recent discussion concerning the simultaneous occurrence of pulmonary tuberculosis and bronchogenic carcinoma suggests a high frequency of coexistence of the two diseases. However, the coexistence of active tuberculosis with bronchiolo-alveolar cell carcinoma, as in our cases, is rare.     

Centrilobular Opacities in the Asthmatic Lung Successfully Treated with Inhaled Ciclesonide and Tiotropium: With Assessment of Alveolar Nitric Oxide Levels

BackgroundDespite the fact that bronchioles are involved in asthma, there have been limited asthmatic cases showing marked centrilobular opacities on computed tomography (CT) chest scans. Systemic corticosteroids have been administered in such cases, but the efficacy of extra-fine particle inhaled corticosteroids has not been assessed.Case SummaryA previously healthy 64-year-old man presented with a four-month history of productive cough and progressive dyspnea despite a combination therapy with inhaled salmeterol (50 μg bid) and fluticasone (500 μg bid), sustained-release theophylline, and pranlukast because of suspicion of asthma. Physical examination revealed wheezing at the end of forced expiration. High resolution CT chest scan showed diffuse centrilobular opacities, bronchiectatic changes, and bronchial wall thickening. Transbronchial lung biopsy, bronchoalveolar lavage fluid, and transbronchial biopsy all showed predominant eosinophil infiltrates, suggesting that eosinophilic inflammation across the entire airway tree caused the abnormal CT findings. Alveolar fraction of exhaled nitric oxide level, a non-invasive marker of eosinophilic peripheral airway inflammation, was also elevated. Because he refused systemic corticosteroids, inhaled ciclesonide (400 μg bid) and inhaled tiotropium were added on to his current medication under careful observation. His symptoms, pulmonary function and CT findings promptly improved, and he had fully recovered at follow-up.DiscussionExtra-fine particle inhaled corticosteroids could be an alternative approach in centrilobular opacities caused by eosinophilic peripheral airway inflammation.     

A case of eosinophilic pneumonia with acute progressive dyspnea and diffuse small nodular shadows on chest X-ray film

A 52-year-old man was admitted to our hospital with high-grade fever, dry cough and severe dyspnea. Chest X-ray films revealed diffuse small nodular shadows in all lung fields, prominently in the middle and lower lung fields. On examination, peripheral blood eosinophilic leukocytosis and severe hypoxemia were demonstrated. His clinical condition improved during five days after the admission without any therapy but oxygen inhalation. The abnormal shadows decreased in several days, and disappeared completely in thirty days. Bronchoalveolar lavage fluid revealed increased total cell counts, mostly with eosinophils (60%) and lymphocytes (21%). Histologically, the transbronchial lung biopsy specimen showed that the walls of pulmonary arteries and bronchioli were markedly infiltrated with eosinophils, and that alveolar septa were edematous with mononuclear and eosinophilic cells infiltration. There were no prominent changes in the alveolar lumen except few macrophage exudates. Our case was of importance for two reasons: first, eosinophilic pneumonia could take the form of interstitial pneumonia both roentgenologically and histologically; second, Crofton's classification of P.I.E. could not be applied to this case.     

Eosinophilic ascites as an uncommon presentation of eosinophilic gastroenteritis: A case report

BackgroundEosinophilic gastroenteritis (EGE) is defined by the presence of gastrointestinal symptoms, with an abnormal eosinophilic infiltrate of the intestine wall and exclusion of other causes of secondary eosinophilia. EGE has three clinical presentations, depending on the depth of eosinophilic infiltration of the bowel wall. It individualizes into three types, namely mucosal, muscular, and subserosal. Eosinophilic ascites, which is caused by edema and eosinophilic inflammation of the serosal layer of the small bowel wall, is the most uncommon presentation of EGE.Case summaryA 30-year-old Egyptian woman presented with pain in the epigastrium and diffuse abdominal distension. Past medical history comprised allergy to iron injections (for iron deficiency anemia). Clinical examination showed moderate abdominal distention (palpation) and shifting dullness (percussion) suggestive of moderate ascites; mild right pleural effusion was also suspected, but findings were otherwise unremarkable. Abdominal and pelvic examinations by ultrasound and contrast-enhanced computed tomography showed moderate ascites, mild right pleural effusion, and diffuse thickening of the antrum and small bowel loops. Endoscopy of the upper gastrointestinal tract revealed mild diffuse hyperemia of the esophagus, stomach and duodenum, with no relevant findings in the histopathology of biopsy specimens taken from these sites. Laboratory results showed eosinophilia in the peripheral blood and marked increase of eosinophils in the ascitic fluid. Treatment with corticosteroids resulted in normalization of the laboratory test results, and the ascites resolved within a week of initiation of therapy.ConclusionEosinophilic ascites, characterized by increased eosinophils in peripheral blood and ascitic fluid, showed dramatic response to steroid therapy.     

A case of desquamative interstitial pneumonia (DIP) suggesting a relationship to chronic eosinophilic pneumonia (CEP)

A 43-year-old man was admitted to our hospital because of cough and dyspnea. His chest X-ray showed bilateral basal infiltrative shadows. The increased eosinophils in BAL suggested chronic eosinophilic pneumonia (CEP), whereas the TBLB specimens showed findings compatible with desquamative interstitial pneumonia (DIP). The open lung biopsy specimens showed typical findings of DIP with a peripheral zone, suggestive of CEP. These two diseases may be only phasal differences of reaction to the same extrinsic stimulation.     

Chronic bronchiolitis with associated eosinophilic lung disease (eosinophilic bronchiolitis).

We encountered an adult patient with dyspnea, eosinophilia and bronchiolitis. He was diagnosed as having diffuse panbronchiolitis, and was treated with erythromycin for 3 years, but his symptoms had gradually worsened. Bronchoalveolar lavage showed marked eosinophilia, and video-assisted thoracoscopic lung biopsy revealed chronic bronchiolitis with associated eosinophilic lung disease (eosinophilic bronchiolitis). To our knowledge, no case of eosinophilic bronchiolitis has been reported in the literature.     

A case of Goodpasture's syndrome with IgA antibasement membrane antibody

This is a very rare case report of Goodpasture's syndrome with IgA antibasement membrane antibody. A 43-year old male was admitted because of severe dyspnea with slight hemoptysis. Chest X-ray demonstrated extensive bilateral infiltrates with air bronchogram, predominantly in the right lung. Laboratory data on admission showed severe anemia and moderate renal impairment. The pulmonary infiltrates resolved spontaneously within 10 days. Goodpasture's syndrome or collagen vascular disease was suspected and he underwent a percutaneous renal and transbronchial lung biopsy. The renal biopsy showed crescent formation affecting 70-80% of glomeruli. Linear IgA deposits, but not IgG, were demonstrated along the glomerular basement membrane by the direct immunofluorescence procedure. The lung biopsy contained many hemosiderin-laden macrophages in the lumen of the alveoli and showed mild thickening of alveolar walls. However, linear immunoglobulin deposits on the alveolar capillary basement membrane were not demonstrated by direct immunofluorescence. The diagnosis of Goodpasture's syndrome with IgA antibasement membrane antibody was made. His serum was negative for antibasement antibody by indirect immunofluorescence. He was treated with prednisone, 30 mg daily. His pulmonary symptoms and anemia improved markedly, but his renal function did not change. Thirteen months after his first admission, he suffered from severe bacterial pneumonia, which was complicated by disseminated intravascular coagulation. He died of respiratory failure. Autopsy was rejected.     

A case of pulmonary eosinophilic granuloma with three-day fever]

A 45-year-old man was admitted to our hospital with chief complaint of fever. The chest X-ray examination showed 2-3 mm fine nodular shadows throughout the entire lung fields. Eosinophilia was present in the peripheral blood. Spike-like high fever (39 degrees C) appeared every 48 hours. All bacteriologic cultures from blood, bone marrow aspirate, sputum, gastric juice, and bronchoalveolar lavage fluid were negative. Furthermore, the antibody titer of malaria was negative. No antibiotics were effective in this case. Histological examination of the transbronchial lung biopsy showed non-specific inflammation, but the open lung biopsy specimen showed scattering of tiny granulomatous lesions. These granulomas were composed of histiocytes with indented nuclei, fibroblasts, varying amounts of collagen fibers, and eosinophils. The histiocytes were positive for S-100 protein staining and identified as Langerhans' cells. Therefore, this patient was diagnosed as having pulmonary eosinophilic granuloma. Two months later, the abnormal shadows on the chest X-ray and high fever spontaneously resolved without steroid therapy. This case was considered to be unique with respect to the peripheral blood eosinophilia, the three-day fever, and spontaneous improvement, compared with the former reported case.     

RAPIDLY PROGRESSIVE FATAL PULMONARY INFILTRATION BY LYMPHOMA

Abstract: :Two patients with diffuse lymphoma, one diffuse large cell and the other diffuse mixed, large and small cell, developed an illness characterised by a high swingeing fever and a pulmonary infiltrate. In both patients there was clinical evidence of chemotherapy-induced tumour response elsewhere at the time lung infiltrates progressed. Blood and sputum cultures, bronchial washings, and, in one case, trans-bronchial lung biopsy did not establish a diagnosis and there was progressive clinical deterioration. Post mortem examination showed widespread involvement of both lungs with lymphoma. The differential diagnosis of fever and pulmonary infiltrates in patients with diffuse lymphoma is discussed, in particular the possibility of this being due to rapidly progressive lymphom     

Pulmonary involvement, pleural effusion, and electrocardiographic abnormality in hypereosinophilic syndrome]

A 47-year-old man was admitted to our hospital with complaints of cough and shortness of breath. Chest radiography showed infiltration of the right lung and left pleural effusion, the eosinophil count increased notably in the peripheral blood, sputum, and pleural effusion. Transbronchial lung biopsy revealed the invasion of eosinophils like eosinophilic pneumonia. Heart failure easily developed in this patient after the intravenous infusion. Myocardial involvement was suspected, and hypereosinophilic syndrome was diagnosed. After prednisolone was administered, the peripheral blood eosinophil count normalized rapidly, and subsequently, the pleural effusion and infiltration shadows in the lung disappeared.     

Eosinophilia and cough induced by resumption of cigarette smoking in a beginning smoker recovering from acute respiratory failure]

A 23-year-old man was admitted to the hospital because of acute respiratory failure accompanied by high fever and severe cough. He had started smoking about 3 weeks earlier and had gradually increased the number of cigarettes he smoked each day. After the number of cigarettes reached 10 per day, the patient experienced cough with serous sputum, high fever (38 degrees C) and dyspnea. Chest X-ray films revealed diffuse, peripheral shadows predominantly in the right lung. The patient was treated with an intravenous drip infusion of antibiotics (FMOX). On the second day of hospitalization, the dyspnea and chest X-ray shadows rapidly resolved. Although the cause of respiratory failure and diffuse shadows on chest X-ray films remained unclear, the patient was discharged. However, on the day of discharge, he smoked cigarettes and experienced cough with serous sputum for a few hours the following morning. He was readmitted for evaluation. We previously reported a case of smoking-induced acute eosinophilic pneumonia (AEP). We suspected that the present case was AEP induced by cigarette smoking but did not perform bronchoalveolar lavage or transbronchial lung biopsy for a definitive diagnosis. Although we did perform a provocation test, the patient demonstrated no respiratory symptoms without cough and respiratory failure. Blood gas analysis and pulmonary function tests also revealed no abnormalities. However, the eosinophil count (3066/mu/l) and percentage (42%) in peripheral blood increased after the patient resumed cigarette smoking, and returned to normal ranges after smoking cessation. These results pointed to a correlation between cigarette smoking and eosinophilia. This case suggested that in some patients, AEP may be induced by cigarette smoking, but does not recur after the resumption of smoking.     

Follicular bronchiolitis associated with pulmonary tuberculosis--report of a case]

The patient, a 39-year-old man, presented in May 1997 with an untreated persistent cough with excessive sputum of 5 years' duration. He was admitted to hospital because of the severity of the symptoms and the presence of acid-fast bacilli in his sputum. These bacilli were identified as Mycobacterium tuberculosis by the polymerase chain reaction method. After treatment with antituberculous drugs was initiated, his symptoms and the patchy infiltrative shadows on his chest radiographs gradually resolved. However, the patient continued to expectorate a purulent sputum, showed diffuse micronodular shadows on chest radiographs, and had hypoxemia as well as mixed ventilatory and small airway disturbances on pulmonary function tests. We performed a video-assisted thoracoscopic biopsy of the lung, which revealed follicular bronchiolitis.     

Two cases of pulmonary Mycobacterium xenopi infection]

We report on two patients diagnosed as having Mycobacterium xenopi infections. Case 1 occurred in a 40-year-old man. His chest radiograph revealed a cavitary lesion with a granular shadow in the right upper lung field. His sputum and bronchial lavage were negative for acid-fast bacilli and malignant cells. For a definitive diagnosis, lung resection was performed by video-assisted thoracoscopy. Case 2 was in a 45-year-old man. His chest radiograph showed a cavitary lesion with infiltration in the right upper lung field. Acid-fast bacilli were seen in his sputum specimens and M. xenopi was identified by culture. Despite medication with isoniazide, rifampicin and ethambutol, the infiltrative shadow in his radiograph increased in size. In this case, right upper lobectomy was performed. In recent years, 8 cases of pulmonary Mycobacterium xenopi infection have been reported in Japan. In the future, the number of such case reports may increase as a result of diagnosis by molecular biological methods. It is necessary to consider carefully whether surgical resection is required when chemotherapy is refused by the patient or is likely to be unsuccessful.     

Juvenile myelomonocytic leukemia (JMML) with the hematologic phenotype of severe β thalassemia

A 3-year-old Filipino-American child with recurrent fever, splenomegaly, anemia, and thrombocytopenia, was found to have a hemoglobin F level of 76.9%. His reticulocyte count was elevated (4.3%), and erythroblasts were present in his peripheral blood. The child's erythrocytes were microcytic (MCV 66.9 fl) but his serum ferritin level was normal. His bone marrow at initial presentation demonstrated normal cellularity without an increase in blast cells. The disease progressed with worsening anemia, leukocytosis, and thrombocytopenia, with increased blasts in his marrow and the appearance of a mediastinal mass. His liver, spleen, and lymph nodes were found to be infiltrated with myeloblasts, supporting a diagnosis of juvenile myelomonocytic leukemia (JMML). Analysis of the child's Hb F showed a ^Gγ/^Aγ ratio of 2.2, which was within the characteristic range for JMML. A globin synthesis study using blood reticulocytes showed an α/non-α globin synthesis ratio of 2.24, typical of severe homozygous β thalassemia. Southern blot analysis of blood-leukocyte DNA from the patient and his parents demonstrated no apparent abnormality in the β-globin gene promoter or coding regions. The elevated level of Hb F in this child with JMML appeared to be part of an acquired Cooley's anemia-like hematologic phenotype. Am. J. Hematol. 58:67–71, 1998. © 1998 Wiley-Liss, Inc.     

A case of eosinophilic pneumonia possibly associated with 5-aminosalicylic acid (5-ASA)]

A 30-year-old man was diagnosed as having ulcerative colitis and was treated with 2,250 mg/day of 5-aminosalicylic acid (5-ASA). After 4 weeks of the administration, the patient complained of cough and fever and was admitted to our hospital. His chest radiograph showed infiltrative shadows bilaterally in the lung fields. Peripheral blood analysis indicated eosinophilia. We confirmed eosinophilic pneumonia by bronchoalveolar lavage and transbronchial lung biopsy. Improvement in clinical symptoms and radiological findings was obtained after the cessation of 5-ASA and initiation of prednisolone. Finally, mesalazine-induced eosinophilic pneumonia was diagnosed on the basis of his clinical course. The literature contains a few reports on patients with mesalazine-induced eosinophilic pneumonia.     

A case of broncho-pulmonary aspergillosis complicated by bronchial asthma attack]

A 55-year-old man was hospitalized for the treatment of severe asthma. However, his condition improved with steroid chemotherapy under artificial ventilation, but high fever and multiple patchy shadows of the lung that were not responsive to antibiotics appeared. We detected aspergillus hyphae, probably inhaled with a quantity of dust in the attic of his workplace, in the sputum. We diagnosed invasive broncho-pulmonary aspergillosis complicated with allergic broncho-pulmonary aspergillosis. His condition improved with anti-fungal drug treatment. We consider that the causes of invasive broncho-pulmonary aspergillosis in this case were massive inhalation of aspergillus conidia, artifical ventilation and steroid chemotherapy.