1/6. dermatomyositis associated with bronchiolitis obliterans organizing pneumonia (BOOP).bronchiolitis obliterans organizing pneumonia (BOOP) is rarely associated with dermatomyositis and may be resistant to conventional corticosteroid therapy under this circumstance. We present a case of BOOP associated with dermatomyositis that responded to a combination of cyclophosphamide and corticosteroid therapy after corticosteroid treatments, alone, had failed. We believe this case shows it is important to recognize that facial rash in the presence of respiratory distress may represent dermatomyositis with BOOP and aggressive treatment may be necessary for resolution of pulmonary symptoms.- - - - - - - - - - ranking = 1keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
2/6. dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia.A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.- - - - - - - - - - ranking = 1.9765804090218keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
3/6. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature.Interstitial pneumonitis may be the presenting manifestation of polymyositis-dermatomyositis, or may occur later in the evolution of the disease. The clinical picture is characterized by non-productive cough, dyspnea and hypoxemia. The chest radiograph demonstrates interstitial infiltrates with predilection for the lung bases, often with an alveolar pattern in addition. The histopathologic features are those of organizing and interstitial pneumonitis and pleuritis, with variable fibrosis. In the present series, the patients with mixed alveolar and interstitial infiltrates on chest radiograph and organizing pneumonia and bronchiolitis obliterans in addition to interstitial pneumonitis. In one patient evolution from pulmonary inflammation to interstitial fibrosis was demonstrated. The etiology of primary lung disease in PM-DM is not known, but cell-mediated autoimmunity to an unidentified component of lung tissue is suggested. Including the present series, 50 percent of patients have responded favorably to corticosteroids with decreased dyspnea, clearing of the chest radiograph and improved pulmonary function tests.- - - - - - - - - - ranking = 0.24707255112772keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
4/6. Interstitial lung disease associated with juvenile dermatomyositis: clinical features and efficacy of cyclosporin A.OBJECTIVES: Interstitial lung disease (ILD) is a rare complication of juvenile dermatomyositis (JDM). The aim of this study was to clarify the clinical features of JDM-associated ILD and to evaluate the efficacy of cyclosporin A (CSA). methods: We reviewed clinical records of 10 cases of JDM that were admitted to Hokkaido University Hospital between April 1990 and March 2001. RESULTS: Five cases were complicated with ILD, three with interstitial pneumonia and two with bronchiolitis obliterans organizing pneumonia. ILD was associated with active JDM and progressed despite corticosteroid therapy. Testing for anti-Jo-1 antibody was negative in all cases. Respiratory symptoms were initially noticed in only one case. In the other cases, ILD was first detected by routine examination of chest X-ray. All the cases received CSA (3-5 mg/kg/day) in combination with prednisolone. One patient died of respiratory failure, but the others responded well to treatment with CSA. CONCLUSION: ILD should be evaluated carefully in all cases of JDM regardless of respiratory symptoms. CSA is a choice for steroid-resistant cases of JDM-associated ILD.- - - - - - - - - - ranking = 0.24707255112772keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
5/6. Inflammatory myopathy, bronchiolitis obliterans/organizing pneumonia, and anti-Jo-1 antibodies--an interesting association.We report an interesting association of inflammatory myopathy, characterized pathologically as dermatomyositis, with bronchiolitis obliterans/organizing pneumonia and anti-histidyl-tRNA synthetase (Jo-1) antibody. The relations of different types of pulmonary involvement to inflammatory myopathy and antisynthetase antibodies are discussed.- - - - - - - - - - ranking = 1.2353627556386keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |
6/6. Rapidly progressive interstitial lung disease associated with dermatomyositis responding to intravenous cyclophosphamide pulse therapy.Interstitial lung disease, especially the rapidly progressive type, carries a grave prognosis when associated with polymyositis (PM)/dermatomyositis (DM). We describe a case of rapidly progressive interstitial lung disease associated with DM. Pathological findings included bronchiolitis obliterans organizing pneumonia (BOOP) pattern in the right upper lung lobe and interstitial fibrosis with microscopic honeycomb lesions in the right lower lung lobe. The patient's respiratory distress was severe and persistent, and oral intubation with mechanical ventilation was transitionally introduced. The respiratory distress condition responded to intravenous cyclophosphamide pulse therapy.- - - - - - - - - - ranking = 0.24707255112772keywords = bronchiolitis obliterans, bronchiolitis, obliterans (Clic here for more details about this article) |