Cases reported "Pulmonary Fibrosis"

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1/152. cyclophosphamide therapy and interstitial pulmonary fibrosis.

    Intersitial pneumonia and pulmonary fibrosis developed in a 72-year-old man during therapy with cyclophosphamide, vincristine, and prednisone. After extensive investigations, including an open lung biopsy, cyclophosphamide appeared to be the cause of the pulmonary disease. Complete disappearance of tachypnea and the pulmonary infiltrates occurred after the discontinuation of cyclophosphamide and the institution of prednisone therapy. We concluded that the diffuse pulmonary disease in this patient was a result of cyclophosphamide therapy. The clinical and pathologic findings in this case and a review of the literature of cyclophosphamide pulmonary toxicity are reported.
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2/152. Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation.

    An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.
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3/152. Coexistent lymphoid interstitial pneumonia, pernicious anemia, and agammaglobulinemia.

    immunologic factors have been incriminated in the pathogenesis of lymphoid interstitial pneumonia. The discovery of a patient with coexistent lymphoid interestitial pneumonia, pernicious anemia, and common variable hypogammaglobulinemia focused attention on the possible autoimmune nature of this pulmonary disease. Extensive immunologic studies demonstrated a noticeably impaired bonemarrow-dependent (B cell) system and intact thymus-dependent (T cell) system. No evidence of humoral or cellular hypersensitivity to homologous lung determinants was found.
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4/152. The diagnosis of brain depression in the presence of severe multisystem disease--a case study.

    Brain depression in a patient with severe multisystem disease can be a diagnostic challenge, particularly when the patient is maintained on artificial life-support systems. A case report is presented of a 13-year-old girl with severe pneumonia who was treated with prolonged cardiopulmonary bypass during which time she developed a clinical picture simulating brain death with marked depression of cerebral cortical activity on two successive EEGs. Following correction of some of her metabolic defects, the patient showed marked improvement of cortical function. Multisystem disease can be so severe as to produce a clinical picture of brain death. We wish to emphasize that brain hypofunction of depression is best evaluated by both clinical examination and the EEG, and that neither one alone is sufficient to conclude that cerebral death has occurred.
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5/152. Good's syndrome presenting with cytomegalovirus pneumonia.

    A 61-year-old woman who had undergone an operation for thymoma 17 years previously suddenly became dyspneic and showed bilateral pulmonary infiltrates on a chest radiograph. In the bronchoalveolar lavage fluid cells contained characteristic cytomegalic inclusion bodies, as well as cytomegalovirus dna demonstrated by a polymerase chain reaction. Immunological findings included hypogammaglobulinemia, deficient numbers of circulating B cells, and impaired blast transformation of peripheral blood T cells in response to mitogens in vitro. Considering all of the findings, the patient was diagnosed with Good's syndrome presenting with cytomegalovirus pneumonia.
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6/152. pulmonary fibrosis induced by cyclophosphamide.

    OBJECTIVE: To report a case of pulmonary fibrosis resulting from use of cyclophosphamide as chemotherapy to treat a patient with breast cancer. CASE SUMMARY: We describe the case of a 52-year-old woman with breast cancer who developed pulmonary fibrosis after four cycles of chemotherapy that included cyclophosphamide. Pulmonary function tests revealed the presence of a severe ventilatory restriction. The open lung biopsy revealed pulmonary fibrosis with vascular sclerosis and signs of pulmonary hypertension. DISCUSSION: cyclophosphamide is an alkylating agent that has been associated with interstitial pneumonia and pulmonary fibrosis. The frequency of these unwanted effects is <1%. The clinical picture consists of the progressive appearance of dyspnea and a non-productive cough that progresses to severe pulmonary insufficiency. The risk factors described for these complications have been the use of chemotherapy regimens that include other drugs with known pulmonary toxicities, the cumulative total dose, the addition of radiotherapy, and the use of high doses of cyclophosphamide. CONCLUSIONS: Even though the frequency of pulmonary fibrosis in patients treated with cyclophosphamide-based chemotherapy regimens is low, the presence of dyspnea and an interstitial pattern in a patient makes it necessary to consider that possible drug toxicity. The open lung biopsy is the most accurate diagnostic technique for these cases. The discontinuation of cyclophosphamide and treatment with corticosteroids is usually followed by clinical recovery in approximately 50% of patients and, in some cases, reversal of the lung injury.
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7/152. polymyositis, pulmonary fibrosis and malignant lymphoma associated with hepatitis c virus infection.

    polymyositis has been associated with various viral infections, and a spectrum of immune-related diseases may occur with hepatitis c (HCV) infection. Both polymyositis and HCV infection may be accompanied by pulmonary fibrosis. An association between polymyositis and malignancy has also been reported. We report a 55-year-old woman accompanied cryoglobulinemia with HCV infection and manifesting polymyositis, pulmonary fibrosis and malignant lymphoma. Steroid therapy was effective to improve interstitial pneumonia, polymyositis, and liver function.
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8/152. Respiratory disease caused by synthetic fibres: a new occupational disease.

    Seven patients exposed to the inhalation of synthetic fibres presented with various bronchopulmonary diseases, such as asthma, extrinsic allergic alveolitis, chronic bronchitis with bronchiectasis, spontaneous pneumothorax, and chronic pneumonia. The histological features are described and an attempt has been made to set up immunological techniques for the diagnosis. A series of histochemical techniques, based on textile chemistry, are proposed for the identification of the inclusions found in bronchopulmonary lesions. The results of the experimental production of the disease in guinea-pigs by the inhalation of synthetic fibre dusts are presented. The prognosis of these cases is good in the acute or recently established cases but is poor when widespread and irreversible fibrosis has set in. The authors consider that pulmonary disease due to inhaled particles is probably set off by an individual factor, possibly immunological.
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9/152. Progressive interstitial fibrosis of the lung in sclerodermoid chronic graft-versus-host disease.

    Sclerodermoid chronic graft-versus-host disease (sGVHD) is a well-known complication in patients with a long history of chronic GVHD. Pulmonary involvement in chronic GVHD presents typically as bronchiolitis obliterans (BO). pulmonary fibrosis after allogeneic hematopoietic stem cell transplantation (HSCT) is presumed to be caused by the long-term toxicity of the conditioning regimen or the result of lung injury elicited predominantly by viral infections or GVHD. We present two patients with late onset pulmonary fibrosis associated with moderate sGVHD of the skin after HSCT. At the initial diagnosis of chronic GVHD both patients presented with symptoms of interstitial pneumonia. Years later both patients developed moderate to severe interstitial pulmonary fibrosis in association with sGVHD. One patient showed additional clinical and histological signs of BO. While one patient responded to increased immunosuppression including total nodal irradiation (1 Gy), the other patient died due to complications related to pulmonary fibrosis.
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10/152. Migratory pulmonary infiltrates in a patient with rheumatoid arthritis.

    The case history is described of an elderly man with rheumatoid arthritis receiving treatment with sulfasalazine and the cyclooxygenase-2 inhibitor celecoxib who presented with severe shortness of breath, cough, and decreased exercise tolerance. The chest radiograph showed unilateral alveolo-interstitial infiltrates and a biopsy specimen of the lung parenchyma showed changes consistent with acute eosinophilic pneumonia. Antibiotic treatment was unsuccessful, but treatment with steroids and discontinuation of sulfasalazine and celecoxib resulted in a marked clinical improvement confirmed by arterial blood gas analysis. The condition may have developed as an adverse reaction either to sulfasalazine or to celecoxib, although hypersensitivity to the latter has not previously been reported.
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