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1/297. 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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keywords = lung
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2/297. Pleural perforation of an aspergilloma cavity occurring in a patient with interstitial lung disease.

    An aspergilloma is a fungus ball resulting from colonization of pre-existing pulmonary cavities, which usually represents a non-invasive form of aspergillosis. Spontaneous rupture of the cavity containing the fungi into the pleural space is an unusual complication that has been reported occasionally in patients with leukemia and invasive aspergillosis. We report on this unusual complication occurring in a patient with underlying interstitial lung disease, in whom the aspergilloma cavity abruptly ruptured into the pleural space with subsequent hydropneumothorax and pleural spillage of the fungi.
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ranking = 5
keywords = lung
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3/297. 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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keywords = lung
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4/297. Nylon flock-associated interstitial lung disease.

    A work-related interstitial lung disease has been diagnosed in workers at five nylon flock facilities in three different states and a Canadian province. The National Institute for Occupational safety and health hosted a workshop at which consulting pulmonary pathologists reviewed lung tissue samples from all the cases for which lung biopsy material was available (15 of 20 cases known in January 1998). After independent review and scoring of these lung tissue specimens, the pathologists reached consensus that the histopathological findings revealed a characteristic lesion-a lymphocytic bronchiolitis and peribronchiolitis with lymphoid hyperplasia represented by lymphoid aggregates. The pathologists noted that the pathological findings were distinctive when compared with known lung conditions. The clinical presentation for the cases generally included cough, dyspnea, restrictive ventilatory defect with reduction in diffusing capacity, and interstitial markings on chest radiographs or high-resolution computed tomography (HRCT) scans. Six of the cases improved after removal from workplace exposure without medical treatment. Six others, who had recovered with medical treatment and removal from the workplace, had relapses in both symptoms and objective findings after attempting to return to nylon flock work. With this and other evidence supporting the existence of chronic interstitial pneumonitis associated with nylon flock processing, workshop participants recommended surveillance for early identification of affected workers and their removal from further workplace exposure.
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ranking = 9
keywords = lung
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5/297. lung adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome.

    We experienced a rare case of lung adenocarcinoma associated with lymphocytic interstitial pneumonitis caused by primary sjogren's syndrome. A 78-year-old woman was referred to our hospital because of progressive sicca syndrome and nodular opacities in the right lower lobe on chest radiograph. This patient was diagnosed as primary sjogren's syndrome by a labial gland biopsy and classical clinical features including xerophthalmia, xerostomia and immunoserological findings. Pathological findings including immunohistochemical studies in a surgically resected lung revealed adenocarcinoma in lymphocytic interstitial pneumonitis associated with primary sjogren's syndrome. There was no evidence of malignant lymphoma in the lymph nodes or resected lung tissue. Pulmonary involvement of sjogren's syndrome is now regarded both clinically and histopathologically as a wide spectrum of lymphoproliferative disorders ranging from benign to malignant. However, lung cancer associated with primary Sjogren's syndrome, as in our case, has apparently not been reported previously.
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ranking = 4
keywords = lung
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6/297. pulmonary veno-occlusive disease, antiphospholipid antibody and pulmonary hypertension in an adolescent.

    pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH); Antiphospholipid antibody (APL) is another known cause of pulmonary hypertension, due to recurrent pulmonary thromboembolism. The coincidence of both causes, PVOD and APL, without thromboembolism, in PH has not been reported previously in children. A 12.5-year-old boy presented with a one year history of fatigue. Pulmonary hypertension was diagnosed by echocardiography. Pulmonary function tests revealed a moderate restrictive pattern and elevated granulocytes were detected in bronchoalveolar lavage. An isolated high-titer APL was detected. Open lung biopsy established the diagnosis of PVOD, with no evidence of pulmonary thrombosis, but with accompanying interstitial and alveolar cellular infiltration. We speculate that APL may have played a role in the pathogenesis of PVOD. prednisone improved the symptoms of the interstitial pneumonitis and was stopped; on follow up of 30 months, the patient ist in stable condition on therapy with nifedipin, phenprocoumon and digoxin. CONCLUSIONS: PVOD and APL may be present simultaneously as a rare cause of PH. Interstitial pneumonitis may accompany PVOD and produce the leading symptoms. Open lung biopsy is essential for early establishment of the diagnosis.
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ranking = 2
keywords = lung
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7/297. Rheumatoid arthritis associated with methotrexate-induced pneumonitis: improvement with i.v. cyclophosphamide therapy.

    Pneumonitis is one of the most serious adverse effects associated with low-dose weekly methotrexate (MTX) therapy. Immediate cessation of MTX, and the introduction of oxygen therapy and glucocorticoids usually results in a dramatic improvement in the pulmonary toxicity. We report here a case of MTX-induced pneumonitis in a patient with rheumatoid arthritis (RA). Severe hypoxemia and interstitial infiltration in both lung fields did not respond to the withdrawal of MTX and the administration of oxygen and steroid pulse therapy. When intravenous cyclophosphamide (CYC) pulse therapy was initiated, however, rapid physiologic and radiographic improvement was seen. Our case suggests that CYC treatment may have a beneficial effect on MTX-induced pneumonitis that is resistant to steroid therapy.
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ranking = 1
keywords = lung
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8/297. Pneumomediastinum complicating bleomycin related lung damage.

    We describe two patients who developed spontaneous pneumomediastinum without pneumothorax against a background of bleomycin-induced pulmonary interstitial disease whilst on treatment for metastatic germ cell tumours of the testis. Pneumomediastinum is a rare but recognized complication of bleomycin-induced lung toxicity, which has previously only been described in association with pneumothorax.
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ranking = 5
keywords = lung
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9/297. 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 = 2
keywords = lung
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10/297. Development of nonspecific interstitial pneumonitis associated with long-term treatment of primary pulmonary hypertension with prostacyclin.

    A young woman with primary pulmonary hypertension presented with interstitial lung disease approximately 5 years after successful treatment with IV prostacyclin. The pathology was consistent with nonspecific interstitial pneumonitis and was unresponsive to steroids and immunosuppressive medications. We speculate that further cases of this syndrome may be reported as more patients are living beyond 5 years with prostacyclin.
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ranking = 1
keywords = lung
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