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1/445. 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. ( info)

2/445. dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum.

    We describe two cases of dermatomyositis (DM), which subsequently developed into rapidly progressive fatal interstitial pneumonitis and pneumomediastinum during steroid therapy. Both cases showed the classical cutaneous manifestations of DM, but the muscular symptoms were absent or mild. Both rapidly progressive interstitial pneumonitis and pneumomediastinum can occur in DM showing less inflammatory changes in the muscles. patients with this form should be treated with extreme caution. ( info)

3/445. Computed tomography and pathologic findings in fulminant forms of idiopathic interstitial pneumonia.

    This study describes the computed tomography features of various fulminant forms of idiopathic interstitial pneumonia and to clarify the usefulness of computed tomography in such patients. Computed tomography scans in 19 patients with fulminant forms of idiopathic interstitial pneumonia were reviewed. This study included patients with acute interstitial pneumonia (n = 7), an accelerated form of idiopathic pulmonary fibrosis (n = 2), and an acute exacerbation of idiopathic pulmonary fibrosis (n = 10). Pathologic confirmation of the diagnosis was obtained in all patients. Follow-up computed tomography scans were available for eight patients, and postmortem computed tomography scans were available for three patients. All patients had progressive ground-glass attenuation, consolidation, or both. In patients with an acute exacerbation of idiopathic pulmonary fibrosis, subpleural honeycombing was also seen. Follow-up computed tomography showed a change from ground-glass attenuation to consolidation with distortion. Architectural distortion, traction bronchiectasis, and ground-glass opacity were the prominent features in the initial computed tomography scans obtained more than 7 days after the onset of symptoms, and cystic lesions were seen in follow-up computed tomography obtained more than 1 month after the onset. High-resolution computed tomography was more sensitive than the plain radiographs in the early detection of these entities. Computed tomography examination at the onset of the acute symptoms is useful in classifying these fulminant forms of idiopathic interstitial pneumonia. ( info)

4/445. 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. ( info)

5/445. amiodarone-induced pulmonary toxicity in an adolescent.

    We report amiodarone-induced pulmonary toxicity in an 18-year-old boy who had undergone corrective surgery for tetralogy of fallot 4 years earlier, and was treated with amiodarone because of recurrent malignant postoperative ventricular tachyarrhythmias. Toxicity was recognized on the basis of clinical features, chest X-ray, high-resolution contrast enhanced computerized tomography, and resolution of the findings subsequent to withdrawal of amiodarone and treatment with steroids. Pulmonary toxicity due to amiodarone, as far as we know, has not been reported in children and young adults, and its occurence even in young adults requires wider appreciation. ( info)

6/445. 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. ( info)

7/445. hydroxyurea-induced acute interstitial pneumonitis in a patient with essential thrombocythemia.

    hydroxyurea is a drug widely used to control myeloproliferative disorders, due in part to its relative lack of severe side effects. We present a case of acute interstitial pneumonitis in a patient who was treated with hydroxyurea for essential thrombocythemia. The clinical course suggests that the interstitial pneumonitis was induced by hydroxyurea. This is the first case of hydroxyurea-induced acute interstitial pneumonitis reported in the literature. ( info)

8/445. 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. ( info)

9/445. Group G streptococcus sacroilitis with sepsis in a 15-y-old adolescent.

    Group G streptococci cause invasive infections of different tissues. Most infected patients have underlying diseases and are of adult age. Invasive group G streptococcal infections rarely occur in childhood and adolescence. A 15-y-old boy with a beta-haemolytic group G streptococcus sacroiliitis, sepsis and secondary pulmonary manifestations resembling an acute respiratory distress syndrome is described. ( info)

10/445. 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. ( info)
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