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1/169. Early assessment of pulmonary involvement in limited scleroderma. A case report.

    Limited scleroderma is typified by insidious progression of skin involvement. The onset of internal organ involvement is delayed until the second decade, the lungs being the most important from the prognostic point of view. Early detection of pulmonary lesions is of paramount importance. This paper presents a 16-year-old male patient with a history of Raynaud's phenomenon followed by progressive tightening of skin over the fingers, hands and face. He had early pulmonary involvement detected by high resolution computed tomography (HRCT) and proven by histopathologic examination as usual interstitial pneumonia; even the chest x-ray and pulmonary function tests were normal. A combination of prednisolone and D-penicillamine was planned for treatment because of his having both pulmonary and gastrointestinal system involvement. 99 m technetium diethylenetriamine pentaacetate (99 m Tc-DTPA) test is very sensitive for pulmonary lesions and it has shown a rapid clearance in the early stage. This method is also useful for following up the therapeutic trial.
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ranking = 1
keywords = pneumonia
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2/169. Computed tomography and pathologic findings in fulminant forms of idiopathic interstitial pneumonia.

    This study describes the computed tomography features of various fulminant forms of idiopathic interstitial pneumonia and to clarify the usefulness of computed tomography in such patients. Computed tomography scans in 19 patients with fulminant forms of idiopathic interstitial pneumonia were reviewed. This study included patients with acute interstitial pneumonia (n = 7), an accelerated form of idiopathic pulmonary fibrosis (n = 2), and an acute exacerbation of idiopathic pulmonary fibrosis (n = 10). Pathologic confirmation of the diagnosis was obtained in all patients. Follow-up computed tomography scans were available for eight patients, and postmortem computed tomography scans were available for three patients. All patients had progressive ground-glass attenuation, consolidation, or both. In patients with an acute exacerbation of idiopathic pulmonary fibrosis, subpleural honeycombing was also seen. Follow-up computed tomography showed a change from ground-glass attenuation to consolidation with distortion. Architectural distortion, traction bronchiectasis, and ground-glass opacity were the prominent features in the initial computed tomography scans obtained more than 7 days after the onset of symptoms, and cystic lesions were seen in follow-up computed tomography obtained more than 1 month after the onset. High-resolution computed tomography was more sensitive than the plain radiographs in the early detection of these entities. Computed tomography examination at the onset of the acute symptoms is useful in classifying these fulminant forms of idiopathic interstitial pneumonia.
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ranking = 8
keywords = pneumonia
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3/169. Acute respiratory distress syndrome in a welder exposed to metal fumes.

    A 43-year-old man began having malaise, chills, and fever 12 hours after cutting a galvanized steel grating with an acetylene torch at work. Over the next 72 hours, his symptoms persisted and became worse with progressive shortness of breath. He was admitted to the hospital and begun on antibiotics and steroids. The next day his condition had deteriorated to the point that he had to be intubated. Chest x-ray film and computed tomography showed patchy and interstitial infiltration bilaterally, consistent with acute respiratory distress syndrome. Open lung biopsy showed focal mild interstitial pneumonia. Multiple laboratory studies were negative for an infectious or an immune process. The patient remained on mechanical ventilation for 10 days and was discharged from the hospital 2 days after extubation. He continued to improve, with minimal symptoms and a return to normal activity levels several months after the incident with no continued treatment. Re-creation of his exposure was done under controlled circumstances, with air sampling revealing elevated air levels for cadmium and zinc and borderline levels of arsenic, manganese, lead, and iron.
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ranking = 1
keywords = pneumonia
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4/169. Interferon gamma (IFN-gamma) deficiency in generalized Epstein-Barr virus infection with interstitial lymphoid and granulomatous pneumonia, focal cerebral lesions, and genital ulcers: remission following IFN-gamma substitution therapy.

    A 26-year-old previously healthy woman developed granulomatous pneumonitis, encephalitis, and genital ulceration during primary Epstein-Barr virus (EBV) infection. EBV dna was demonstrated by polymerase chain reaction analysis of serum, lung tissue, and genital ulcer specimens. serology verified primary EBV infection. The patient lacked lymphocytes cytotoxic to autologous EBV-transformed B lymphocytes. No spontaneous or in vitro EBV-induced interferon gamma (IFN-gamma) production was evident in peripheral blood. The cells had normal IFN-gamma production when stimulated with staphylococcus aureus exotoxin A. In the bone marrow and peripheral blood, the number of large granular CD56 lymphocytes (natural killer cells) increased 39%-55%, but no CD4 or CD8 cell lymphocytosis was initially found. A partial clinical response was achieved with treatment with acyclovir, corticosteroids, and intravenous gamma-globulin. Because of persistent granulomatous central nervous system and lung involvement, subcutaneous IFN-gamma therapy was started but was discontinued after 3 months because of development of fever, pancytopenia, and hepatitis. This therapy initiated a complete clinical recovery, which occurred parallel to development of EBV-specific cytotoxic CD8 T lymphocytes and normalization of natural killer cell lymphocytosis. These findings provide evidence for an EBV-induced lymphoproliferative disorder due to a T lymphocyte dysfunction associated with a selective lack of IFN-gamma synthesis.
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ranking = 4
keywords = pneumonia
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5/169. Infantile pulmonary alveolar proteinosis with interstitial pneumonia: bilateral simultaneous lung lavage utilizing extracorporeal membrane oxygenation and steroid therapy.

    An infant with refractory pulmonary alveolar proteinosis (PAP) associated with severe interstitial pneumonia is described. Although she was treated by bilateral simultaneous lung lavage utilizing extracorporeal membrane oxygenation and steroid therapy, she died of progressive respiratory failure 28 days after admission. Histologic examination of lung autopsy specimen showed only partial alveolar spaces to be filled with a dense PAS positive granular eosinophilic material and showed severe interstitial pneumonia with marked fibrosis of alveolar walls and interstitium. The lung lavage seemed to be effective for PAP because the effluent fluid sufficiently became clear and the PAS positive material was detected only in partial alveoli. The full venoarterial cardiopulmonary bypass with extracorporeal membrane oxygenation seemed to be very useful to support bilateral lung lavage for small infants. The refractory symptoms and failure of treatment were resulted from the association of severe interstitial pneumonia. In neonates or infants with PAP and severe interstitial pneumonia with poor response for steroid therapy, the lung transplantation should be considered.
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ranking = 8
keywords = pneumonia
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6/169. Interstitial pneumonitis following mitozantrone, chlorambucil and prednisolone (MCP) chemotherapy.

    We describe two cases of interstitial lung disease in patients with non-Hodgkin's lymphoma who were treated with combination chemotherapy including mitozantrone. In both we had radiological and histological evidence of interstitial lung disease, with patterns of organizing pneumonia and hypersensitivity without a clear aetiological agent. Clinical resolution occurred on withdrawal of chemotherapy. One patient required a course of corticosteroid treatment. To date, both patients are well and in remission, and there has been no recurrence of their respiratory disease. We postulate that these observations are hitherto undescribed pulmonary abnormalities secondary to mitozantrone therapy.
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ranking = 1
keywords = pneumonia
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7/169. A case of group II non-specific interstitial pneumonia developed during corticosteroid therapy after acute respiratory distress syndrome.

    Non-specific interstitial pneumonia/fibrosis (NSIP) is a relatively new entity among idiopathic interstitial pneumonias. This disease is pathologically characterized by varying proportions of interstitial inflammation and fibrosis that appear to occur over a single, short period of time, and is clinically characterized by a generally good prognosis. Although corticosteroid therapy is widely considered to be effective for treatment of this disease, we report a case of Group II NSIP which developed during treatment with corticosteroid 4 months after acute respiratory distress syndrome (ARDS). This case suggests that any group of NSIP may be developed after ARDS, and that low-dose corticosteroid treatment is not sufficient to prevent the development of NSIP.
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ranking = 6
keywords = pneumonia
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8/169. breast cancer shortly after the diagnosis of interstitial pneumonia associated with polymyositis.

    polymyositis is sometimes associated with pulmonary fibrosis or malignant diseases, however, rarely with both simultaneously. For such patients, no criterion for initial treatment and tapering of steroid has been reported. We describe a patient who had breast cancer and interstitial pneumonia associated with polymyositis. In this case, slow tapering of the steroid was an important aspect of the treatment, and close clinical follow-up was necessary to monitor for disease exacerbation.
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ranking = 5
keywords = pneumonia
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9/169. Increasing dose of methylprednisolone pulse therapy treats desquamative interstitial pneumonia in a child.

    A 10 yr old male with hypoxaemia, progressive infiltration on the chest radiograph and biopsy-proven desquamative interstitial pneumonia was treated with daily oral prednisolone for 6 months. Intravenous methylprednisolone pulses were concomitantly administered in doses averaging 10 mg x kg body weight(-1) on three consecutive days every 4-6 weeks. After 6 months improvement could be noted and oral steroids were stopped, while pulse therapy continued. Three months later, when seven pulses had been administered, a relapse occurred and the clinical status deteriorated. Instead of reinstating daily systemic steroids, the dose of methylprednisolone pulses was increased to 20 mg x kg body weight(-1) i.v. given on three consecutive days and repeat pulses every 4 weeks. This was followed by continuous improvement. After 24 months corticosteroid pulses were terminated. Normal lung function, serum lactate dehydrogenase, blood gases upon exertion and regular development was achieved. During the course of treatment, the child has grown 10 cm. It is concluded that the effect of corticosteroid pulse therapy on interstitial lung disease in childhood is dose-dependent and that the dose can be adjusted to the effect observed.
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ranking = 5
keywords = pneumonia
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10/169. Flock worker's lung: broadening the spectrum of clinicopathology, narrowing the spectrum of suspected etiologies.

    STUDY OBJECTIVES: Workers in the nylon flocking industry recently have been found to be at increased risk of chronic nongranulomatous interstitial lung disease. Although a spectrum of cytologic and histopathologic abnormalities has been observed, nonspecific interstitial pneumonia, lymphoid nodules, and lymphocytic bronchiolitis predominated in the 19 previously reported cases of flock worker's lung. Here we describe five additional patients who appear to expand the histopathologic spectrum and add to the evidence suggesting a causative role for respirable-sized nylon fragments. methods: We studied all North American patients (n = 5) found in 1998 to satisfy our previous case definition of flock worker's lung. Two pulmonary pathologists independently reviewed each biopsy specimen. RESULTS: All five patients reported cough and dyspnea. Only one patient had crackles on chest auscultation. High-resolution CT scan, interpreted with attention to subtle ground-glass attenuation, remained a highly sensitive diagnostic test. Pulmonary function tests and plain chest radiograph were less sensitive. One patient's wedge biopsy showed previously described prototypical findings. Two others had transbronchial biopsies showing some of the same features. The fourth patient's wedge biopsy showed desquamative interstitial pneumonia. The fifth patient had bilateral synchronous adenocarcinoma but with radiographic evidence of diffuse interstitial fibrosis. These 5 patients and the 19 patients studied previously were exposed to nylon flock manufactured by a rarely used cutting technology. CONCLUSION: Findings in these five patients appear to broaden the spectrum of the clinicopathology of flock worker's lung and add to the evidence incriminating respirable-sized nylon particulates produced during the manufacture and use of rotary-cut nylon flock.
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ranking = 2
keywords = pneumonia
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