Cases reported "Pulmonary Fibrosis"

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1/16. Superior vena cava obstruction caused by radiation induced venous fibrosis.

    superior vena cava syndrome is most often caused by lung carcinoma. Two cases are described in whom venous obstruction in the superior mediastinum was caused by local vascular fibrosis due to radiotherapy five and seven years earlier. The development of radiation injury to greater vessels is discussed, together with the possibilities for treatment of superior vena cava syndrome.
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2/16. microscopic polyangiitis presenting as idiopathic pulmonary fibrosis: is anti-neutrophilic cytoplasmic antibody testing indicated?

    We report a 55-year old woman with microscopic polyangiitis who presented with idiopathic pulmonary fibrosis and 1 year later developed hematuria and proteinuria. She had a high serum level of perinuclear anti-neutrophilic cytoplasmic antibodies. Renal angiogram was normal. The diagnosis of microscopic polyangiitis was confirmed by renal biopsy, which showed pauci-immune crescentic glomerulonephritis. The patient received immunosuppressive therapy and improved markedly. Consideration of small vessel vasculitis is important in the differential diagnosis of idiopathic pulmonary fibrosis.
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3/16. Pulmonary fibrosis and other clinical manifestations of small vessel vasculitis in a family with seropositive juvenile rheumatoid arthritis.

    We report on a family (mother, daughter, and son) suffering from progressive pulmonary fibrosis associated with deforming arthritis, sinusitis, glomerulonephritis, and cutaneous vasculitis. We suggest that these clinical features display the variable expressions of small-vessel vasculitis in juvenile rheumatoid arthritis.
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4/16. Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: successful treatment with endovascular stent implantation.

    A variety of pulmonary vascular disorders, such as hemangiomatosis, telangectasia, and veno-occlusive disease, may be involved in the pathogenesis of interstitial lung diseases. We describe the case of a girl with recurrent bacterial pneumonia and progressive interstitial fibrosis affecting the right lung. Morphologic evaluation of the lung biopsy showed structural changes of the vessel walls suggesting pulmonary hypertension. The echocardiogram showed the presence of centripetal blood flow in the right pulmonary artery from the periphery of the lung to the heart. A selective right angiography demonstrated the presence of pulmonary venous obstruction at the veno-atrial junction, successfully treated by endovascular stent implantation during cardiac catheterization.
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5/16. Pulmonary interstitial fibrosis as a presenting manifestation in perinuclear antineutrophilic cytoplasmic antibody microscopic polyangiitis.

    microscopic polyangiitis (MPA) is one of the vasculitides that is included in the pulmonary renal syndromes. Pathologically, MPA has been defined as necrotizing vasculitis with few or no immune deposits, primarily affecting small vessels including arterioles, venules, or capillaries. Pulmonary interstitial fibrosis (PIF) as an accompanying manifestation in MPA has not been widely appreciated. In the present study, we report six cases of MPA at our institution with radiographic evidence of PIF that was apparent before any treatment was administered. All had biopsy evidence of renal disease that was consistent with MPA as well as positive serum perinuclear antineutrophilic cytoplasmic antibody titers. hemoptysis was observed in approximately one half of the patients. As determined by CT of the chest, PIF was detected in all of the patients and was often present years before a diagnosis of MPA was made. We conclude that PIF may occur as a pulmonary manifestation of MPA. Further appreciation of this finding may lead to more data with respect to the incidence of PIF in MPA, and to a better understanding of the mechanisms that are involved in the development of this finding.
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6/16. Acute eosinophilic pulmonary disease associated with the ingestion of L-tryptophan-containing products.

    A series of four patients with pulmonary infiltrates, pleural effusions, hypoxemia, peripheral eosinophilia, and symptoms of dyspnea, fatigue, and weakness is reported. Lung tissue obtained in three patients revealed interstitial pneumonitis, small-to-medium-vessel mixed-cell vasculitis, and alveolar exudate of histiocytes and eosinophils. All patients reported ingestion of L-tryptophan-containing products at a time when an association between L-tryptophan and the eosinophilia-myalgia syndrome was established. This clinical pattern of pulmonary involvement may be part of the continuum of the eosinophilia-myalgia syndrome. The pathophysiology of this syndrome and the relationship with the ingestion of L-tryptophan-containing products have not yet been identified.
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7/16. Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients.

    Between 1978 and 1988, three patients at our institution had an initial diagnosis of idiopathic pulmonary fibrosis but later were correctly diagnosed as having pulmonary-renal syndrome and microscopic polyarteritis. These cases involved elderly patients with progressive dyspnea and nonproductive cough, bilateral dry crackling rales, bilateral interstitial infiltrates evident on a chest roentgenogram, and restrictive findings on pulmonary function testing. In two patients, lung biopsy specimens were obtained, and an initial diagnosis of nonspecific pulmonary fibrosis was made. All three patients eventually had microhematuria and renal insufficiency. A revised diagnosis of small-vessel pulmonary-renal vasculitis was based on the demonstration of segmental necrotizing glomerulonephritis in renal biopsy specimens in two patients, thrombotic microangiopathy consistent with healed vasculitis on postmortem examination of the kidney in one patient, and subsequent detection of small-vessel vasculitis on review of the two lung biopsy specimens. Anti-neutrophil cytoplasmic antibodies with perinuclear staining on indirect immunofluorescence microscopy were positive in the two patients in whom determinations were performed. The clinical manifestations of vasculitis were notably scarce--no involvement of the skin, nervous system, or gastrointestinal tract; no episodes of fever; and minimal or absent musculoskeletal symptoms. These cases illustrate the importance of a high index of suspicion for the diagnosis of systemic vasculitis in elderly patients and the need to consider a vasculitis in the differential diagnosis of idiopathic pulmonary fibrosis, especially if an active urinary sediment is present.
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8/16. Lung fibrosis induced by Thorotrast.

    A 63 year old woman developed progressive shortness of breath, pulmonary hypertension, and respiratory failure and died from pulmonary fibrosis 45 years after thoracic fistulography with Thorotrast. Bouts of acute respiratory failure occurred with features of noncardiogenic pulmonary oedema. Lung tissue obtained by biopsy and at necropsy showed abundant radioactive particles of thorium dioxide in the lungs. The particles were congregated in the walls of blood vessels and in perivascular fibrous zones, consistent with a causal role of Thorotrast in the development of lung fibrosis. It is suggested that the fibrosis was due to the combined effects of alpha radiation on the interstitial perivascular zones and of recurrent pulmonary oedema due to endothelial damage.
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9/16. Thoracic hemangiomatosis masquerading as interstitial lung disease.

    Pulmonary capillary hemangiomatosis is an extremely rare condition, first described by Wagenvoort. In this condition, sheets of thin-walled blood vessels infiltrate the lung parenchyma, bronchioles, and pleura. Three previous cases have been described in the literature. We describe a fourth. It probably represents a congenital abnormality.
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10/16. Pseudomembranous colitis in a patient of paraquat intoxication.

    A 49 year-old man drank paraquat 7-8 fold of human fatal dose, and died of severe respiratory failure in 24 days in spite of intensive therapy. The autopsy revealed diffuse pulmonary fibrosis caused by prolonged intoxication of paraquat. An interesting finding was many raised plaques from 1 mm to 10 mm in diameter, observed in whole colon. Histologically, these plaques were composed of necrotic mucosa ("pseudomembrane"), disrupted crypts and edematous submucosa with fibrin education, intervening in normal colon tissue with sharp margins, and diagnosed as non-antibiotic-induced pseudomembranous colitis. Since the edematous submucosa of the raised plaque had a stratified fibrin education around a dilated capillary, it was speculated that the vessel injury by paraquat might play a role in the pathogenesis of pseudomembranous colitis.
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