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1/27. 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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2/27. Clinical features of three fatal cases of non-specific interstitial pneumonia.

    We describe the clinical courses of the 3 fatal patients (2 females and 1 male) with idiopathic non-specific interstitial pneumonia (NSIP) among 24 patients with NSIP. lung biopsies were diagnosed to be NSIP group II in all patients. The clinical courses from onset to death of these 3 patients were 41 months, 46 months, and 91 months. A follow-up chest CT demonstrated no apparent honey-comb formation. We found that i) about 20% of patients with NSIP died of respiratory failure, ii) in the chest CT findings, apparent honey-comb formation was rare even just before death, iii) prediction of the prognosis based on the histological findings was difficult. This is the first report to describe the clinical features of deceased patients with idiopathic NSIP; the incidence of fatal cases was considered to range from 10 to 20%.
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3/27. Respiratory bronchiolitis associated interstitial lung disease (RB-ILD) presenting with haemoptysis.

    Respiratory bronchiolitis associated interstitial lung disease is an uncommon condition in current or ex-smokers. The presentation is non-specific, but haemoptysis is uncommonly reported in this condition. We report the case of a 25-year-old woman who presented with significant haemoptysis, dyspnoea, reduced transfer factor and normal clinical examination. In addition, a medline literature search was performed to review the clinical features and prognosis of this disease. Other causes of haemoptysis were excluded with extensive investigation. The diagnosis was made on thoracoscopic lung biopsy. The patient had significant postoperative complications of prolonged air leak and hydropneumothorax requiring further surgery and prolonged hospital stay. Advice regarding smoking cessation was given. Her pulmonary physiology remains abnormal on follow up but symptoms have improved. Respiratory bronchiolitis-ILD may present with normal examination and radiology. Haemoptysis in this case may have been associated with the underlying disease but could have been incidental. diagnosis, in general, requires lung biopsy. As in this patient, lung function does not appear to improve significantly on follow up.
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4/27. Interstitial pneumonia accompanying ulcerative colitis.

    We report a patient with ulcerative colitis complicated with idiopatic interstitial pneumonia, in whom the etiology of interstitial pneumonia was unknown, but immunological disturbance might have been involved. There are many complications with ulcerative colitis, but interstitial pneumonia is quite rare and its prognosis is quite poor. Antibiotic and steroid treatment were given under respiration supported therapy, but no response could be obtained. In the treatment of patients with ulcerative colitis, we must be mindful of interstitial pneumonia because the prognosis is quite poor.
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5/27. Severe interstitial pneumonitis associated with docetaxel administration.

    BACKGROUND: Interstitial pneumonitis has not been reported as a toxicity of docetaxel. The authors report the presentation and natural history of four patients who developed a severe interstitial pneumonitis after receiving docetaxel. methods: The hospital and outpatient records of patients treated with docetaxel were reviewed to identify whether any of these patients required an evaluation for respiratory problems. RESULTS: Four patients developed an interstitial pneumonitis that could be explained only as a toxicity of docetaxel. None had metastatic disease to the lung, and all had normal liver function before receiving chemotherapy. The patients presented with acute dyspnea and fever within 1-2 weeks of receiving docetaxel. All developed progressive interstitial infiltrates and respiratory failure that required mechanical ventilation. An exhaustive workup for other causes of pneumonitis was negative. Broad-spectrum antibiotics and corticosteroids were ineffective. Two patients died of complications related to the pulmonary process. The two survivors required ventilatory support for more than 21 days. The clinical and pathologic findings of these patients are presented. CONCLUSIONS: Interstitial pneumonitis is a rare and potentially fatal complication of docetaxel treatment. Prolonged ventilatory support is appropriate in patients with a favorable prognosis.
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6/27. Giant cell interstitial pneumonia in a 15-year-old boy.

    Giant cell interstitial pneumonia (GIP) is a very uncommon respiratory disease. We report a juvenile patient with GIP aged 15 years. Although he has a negative past history of direct exposure to hard metals, we could not exclude possible exposure in very small amounts through his parents. Microscopic examination of lung biopsy specimen obtained by video-assisted thoracoscopy revealed marked cellular interstitial infiltrates and prominent intraalveolar macrophages as well as giant cells showing cellular cannibalism. Analysis of the biopsied lung tissue for cobalt and tungsten was negative. Clinical symptoms, laboratory, and radiological findings improved markedly after treatment with corticosteroids. To our knowledge, only eleven cases of GIP have been reported in japan. Although possible exposure to hard metals was identified in 9 of the 11 reported cases, there is no clear dose-dependent relationship with onset and prognosis. The average age at onset was 46.2 /-15.0 years. Our patient is the youngest case of GIP reported in the world.
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7/27. Acute interstitial pneumonia: report of a series.

    Four cases of acute interstitial pneumonia (AIP) are described with special emphasis on clinical background, lung imaging and bronchoalveolar lavage findings. A retrospective chart review of four patients with histologically-proven AIP, diagnosed between 1998 and 2000, was carried out. Clinical data, bronchoalveolar lavage (BAL) findings, high-resolution computed tomography (HRCT) and histological features were analysed. Three patients died and only one is in follow-up. HRCT showed areas of ground glass attenuation and alveolar consolidation in all patients. histology, documented by open lung biopsy or autopsy specimens, was consistent with the organising form of diffuse alveolar damage pattern. BAL findings were characteristic, with a huge neutrophilia associated with scattered atypical type II pneumocytes collected in clusters with extracellular amorphous material (fragments of hyaline membranes) observed in two out of three cases. In this paper, four cases of acute interstitial pneumonia are reported in detail. The poor prognosis associated with this entity has been confirmed and the possible diagnostic role of the bronchoalveolar lavage is emphasised.
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8/27. Disseminated cutaneous herpes simplex virus type-1 with interstitial pneumonia as a first presentation of AIDS.

    Recognition of cutaneous herpes simplex virus (HSV) in acquired immunodeficiency syndrome (AIDS) can be difficult because of its atypical prsentations. Pneumonia in AIDS is complex with many possible etiologies. Identification of HSV preceded by atypical cutaneous manifestation and pneumonia is critical, given the poor prognosis when treatment is delayed or not prescribed. We report a 62-year-old patient with undiagnosed hiv infection who presented with disseminated cutaneous lesions resembling impetigo as his first presentation of AIDS. Subsequent confirmation that HSV was responsible led to appropriate treatment of this and the complicating HSV pneumonia. This report emphasizes the importance of the early recognition of atypical cutaneous HSV in patients with AIDS.
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9/27. Prevention of progression of interstitial lung lesions by early combination therapy with corticosteroids and cyclosporine/cyclophosphamide in two patients with amyopathic dermatomyositis.

    Two patients with amyopathic dermatomyositis complicated by interstitial lung lesions were effectively treated with a combination of corticosteroids and cyclosporine and/or cyclophosphamide. A 48-year-old female patient was treated with pulse methylprednisolone and cyclosporine 2 months after onset of dermal symptoms. A 45-year-old male patient was treated with oral prednisolone and pulse cyclophosphamide 2 1/2 months after onset of dermal symptoms. Early evaluation of interstitial lung lesions and early extensive therapy may improve prognosis of interstitial lung lesions in patients with amyopathic dermatomyositis.
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10/27. Pulmonary side effects of interferon-alpha therapy in patients with hematological malignancies.

    Several side effects of interferon-alpha-2b (IFN-alpha) therapy have been described. Pulmonary side effects have seldom been reported. The four patients we describe all developed respiratory disorders while being treated with IFN-alpha for hematological malignancies. We point out the similarities and differences noticed concerning the clinical course, pathological findings, and prognosis in the four different cases. Also, in addition to our review of the literature, we discuss the possible mechanisms involved in development of lung symptoms. In three patients the pulmonary disorder seems to have been caused by a cell-mediated immunological side effect in the form of interstitial pneumonitis. In one patient the symptoms were most likely caused by an autoimmunologic reaction, primarily engaging the vascular system, initially in the lungs. The single pathological finding existing in all four cases was the marked decrease in carbon monoxide diffusion capacity when performing spirometry. This was not necessarily associated with the existence of radiological findings. The decrease in diffusion capacity and the clinical symptoms were completely reversible in three of the patients, either spontaneously after the withdrawal of IFN-alpha or after treatment with corticosteroids. Our conclusion is that IFN-alpha, on rare occasions, can cause serious pulmonary side effects when used to treat both lympho- and myeloproliferative hematological malignancies. When pulmonary symptoms are evaluated during IFN-alpha therapy, spirometry, including estimation of carbon monoxide diffusion capacity, high-resolution computerized tomography, and ultracardiography should be used.
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