Cases reported "Pulmonary Fibrosis"

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1/12. 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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2/12. Persistent pneumomediastinum in interstitial fibrosis associated with rheumatoid arthritis: treatment with high-concentration oxygen.

    We present a case of persistent spontaneous pneumomediastinum precipitated by an upper respiratory infection in a patient with interstitial fibrosis associated with rheumatoid arthritis who was receiving chronic corticosteroid treatment. The persistent nature of the mediastinal emphysema over 2 months eventually required treatment with high concentrations of inhaled oxygen that resulted in rapid resolution of the pneumomediastinum without recurrence over 6 months of follow-up. This case, along with others in the medical literature, emphasizes the need for early use of high-concentration inhaled oxygen in the treatment of pneumomediastinum in high-risk patients, such as those with connective tissue disorders.
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3/12. "Blue bodies" in a case of cryptogenic fibrosing alveolitis (desquamative type) an ultra-structural study.

    A patient with cryptogenic fibrosing alveolitis, with both mural and desquamative features, had two lung biopsies at the times of coronary artery surgery. These lung specimens were studied, using light and electron microscopy, with immunofluorescence techniques and electron microanalysis. In addition to the typical changes of cryptogenic fibrosing alveolitis previously reported, we found "blue-staining bodies" within alveolar macrophages and giant cells. These bodies were 15--25 micrometer in diameter with an iron rich outer rim and core of connective tissue mucin--possibly chondroitin sulphate or dermatan sulphate. It seems unlikely that these "blue bodies" were due to fibreglass dust to which the patients had had a trivial exposure, but their exact nature and significance remains unclear.
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4/12. Bronchopulmonary involvement in ulcerative colitis.

    We report two cases of pulmonary involvement in ulcerative colitis. The first patient, a 37-year-old woman, had bilateral basal bronchiectasis full of mucopurulent secretion, with a marked improvement of pulmonary function and roentgenographic appearance after a conservative approach. The second patient had severe pulmonary fibrosis of autoimmune nature and died owing to a pulmonary infection. In the second patient, a sulfasalazine reaction as an etiologic factor was excluded, while in the first this possibility seemed unlikely. Therefore, we take these two cases as examples of the extraintestinal manifestations of ulcerative colitis.
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5/12. cytomegalovirus interstitial pneumonitis in a bone marrow transplant recipient.

    A 15-year-old girl suffering from acute lymphoblastic leukemia developed cytomegalovirus interstitial pneumonitis 34 days after an allogenic bone marrow transplantation. The disease was diagnosed by open lung biopsy. Histopathologic examination disclosed intranuclear and intracytoplasmic inclusion bodies, as well as, a positive cytomegalovirus antigen detected by an immunofluorescence stain using a cytomegalovirus monoclonal antibody. The virus culture also eventually produced cytomegalovirus. Because of the lack of ganciclovir in this country, antiviral therapy of a non-specific nature was given to this patient. However, the treatment was ineffective and she subsequently died. There is an association between the immunologic events of a graft-versus-host disease and the development of cytomegalovirus interstitial pneumonitis. The pathogenesis of cytomegalovirus interstitial pneumonitis in a bone marrow transplant recipient is evidence of an immunopathologic disease, rather than that of a purely infectionus disease. years, the treatment modality of cytomegalovirus interstitial pneumonitis in bone marrow transplant recipients has become a combination of specific antiviral and immune therapy.
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6/12. Computed tomography in interstitial lung disease.

    The number and scope of published articles dealing with computed tomography of interstitial lung disease are limited. We present seven cases in which computed tomography detected the presence or extent of interstitial lung disease better than conventional radiography: two patients with histiocytosis X, one with bronchopulmonary dysplasia, one with bleomycin lung toxicity, and three with radiation-induced lung injury. The computed tomography appearance of histiocytosis X and bronchopulmonary dysplasia have not been previously described. Transverse computed tomography images provide information regarding stage of activity and nature of interstitial lung processes not available with standard imaging techniques. We advocate the use of computed tomography in the initial investigation and follow-up of patients with histiocytosis X. Post-radiation pneumonitis and fibrosis can be detected earlier with computed tomography as well.
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7/12. Unilateral pulmonary fibrosis associated with a contralateral epiphrenic diverticulum--support for a hypothesis.

    A case of predominantly unilateral pulmonary fibrosis associated with a contralateral epiphrenic oesophageal diverticulum is reported. This experiment of nature is offered to support the hypothesis that cryptogenic fibrosing alveolitis (idiopathic pulmonary fibrosis) may in some instances be due to repeated aspiration of gastroesophageal contents.
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8/12. kaolin pneumoconiosis. A case report.

    A 35-year-old man who had been occupationally exposed to aerosolized kaolin for 17 years in a georgia processing plant had diffuse reticulonodular pulmonary infiltrates and an upper lobe mass. Exploratory thoracotomy, performed to evaluate the nature of the mass, revealed an 8 X 12 X 10-cm conglomerate pneumoconiotic lesion containing large amounts of kaolinite. Coincident deposition of silica in the tissue was not demonstrable by either analytic scanning electron microscopy or x-ray diffraction. The case illustrates the effect of chronic kaolin exposure on the human lung and emphasizes the need for periodic evaluation of exposed workers.
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9/12. Mucoepidermoid carcinoma of the thymic region.

    A case of mucoepidermoid carcinoma in thymus in a 59-year-old Japanese female is presented. She died of cardiac tamponade due to tumor invasion ater a 5 years' clinical course. At autopsy the main tumor was found in the thymic region with metastases to the sternum, regional lymph nodes, pericardial, and left pleural cavity. The mucoepidermoid carcinoma might be probably originated from a hen's egg-sized cyst which was located in the upper posterior aspect of the tumor-involved thymus. No teratomatous components were present. The cyst was most likely to be of thymic or bronchogenic cyst origin, though it was not determined, in view of the lining with pseudostratified ciliated columnar epithelium of the cystic wall and the surrounding with the thymic tissue outside. Moreover, there was thymic hyperplasia with germinal center that was compatible with SLE-like symptoms in her past history and autoimmune nature of the autopsy findings of pulmonary fibrosis.
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10/12. lymphomatoid granulomatosis.

    The exact nature of lymphomatoid granulomatosis remains unclear. Whether it represents a viral infection, an autoimmune disorder (possibly induced by a virus), or primary malignant lymphoma, is still to be determined. The histopathology and clinical course allows classification of lymphomatoid granulomatosis as a distinct entity. Unfortunately, there is no consistently effective therapy for this condition and the usual course is rapidly downhill, though temporary remissions may be obtained. Since presentation of these patients with cutaneous lesions is not unusual, dermatologists must be alert to the possibility of an underlying systemic vasculitis in patients with atypical cutaneous ulceration, especially when no obvious cause is apparent.
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