1/26. 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 = pneumonia (Clic here for more details about this article) |
2/26. Hyperimmunoglobulin E-recurrent infection syndrome in a patient with juvenile dermatomyositis.A 13-year-old girl presented with multiple skin abscesses. She was diagnosed as having juvenile dermatomyositis (DM) at the age of 7 years. She had suffered from recurrent skin infections, atypical pruritic dermatitis and pneumonia since the age of 8 years. Bacteriologic and fungal cultures for skin abscesses and oral mucosa were positive S. aureus and C. albicans, respectively. Chemotactic defect in peripheral blood neutrophils was observed. The level of serum IgE was markedly elevated, and anti-S.aureus specific IgE was found. A diagnosis of hyperimmunoglobulin E-recurrent infection syndrome (HIE) was made and she was successfully treated with surgical drainage and antibiotics. To our knowledge, this is the first case report of HIE in a patient with juvenile dermatomyositis.- - - - - - - - - - ranking = 0.2keywords = pneumonia (Clic here for more details about this article) |
3/26. 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 = 1.6keywords = pneumonia (Clic here for more details about this article) |
4/26. Successful combined therapy of cyclophosphamide and cyclosporine for acute exacerbated interstitial pneumonia associated with dermatomyositis.Interstitial pneumonia of polymyositis and dermatomyositis (PM/DM), especially the rapid progressive type, is a serious complication. We report a case of acute exacerbated interstitial pneumonia associated with DM. The respiratory distress condition was refractory to steroid pulse therapy and cyclosporine, however, there was a good response to additional intravenous cyclophosphamide pulse therapy (IVCY). We propose the possibility that the combination of these immunosuppressants might be useful for interstitial pneumonia with DM which is resistant to conventional steroid therapies.- - - - - - - - - - ranking = 1.4keywords = pneumonia (Clic here for more details about this article) |
5/26. A case of dermatomyositis associated with mechanic's hand.A 67-year-old man was referred to the Department of internal medicine at tokyo Medical University with interstitial pneumonia in July 1999. He presented with keratotic plaques on both palsm and on the ventral and lateral sides of his fingers. Erythematous keratosis was observed on the dosal aspect of his fingers and metatarsophalangeal (MP) joints. Edematous erythema was seen on the patient's chest, back, and the extensor surfaces of his arms. electromyography revealed a myogenic pattern and an increased level of myogenic enzymes was found in the blood. Histological findings of the ventral sides of his fingers showed hyperkeratosis and parakeratosis of the dermal tissue and liquefaction degeneration of the basal layer at the papilla. Based on these findings, the patient was given a diagnosis of dermatomyositis associated with mechanic's hand. A systemic examination confirmed interstitial pneumonia and carcinoma of the duodenal papilla. Mechanic's hand is a type of dermatitis associated with myopathy first reported by Stahl et al. in patients with collagen disease. We report herein the first documented case of mechanic's hand in Asians.- - - - - - - - - - ranking = 0.4keywords = pneumonia (Clic here for more details about this article) |
6/26. 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 = 0.2keywords = pneumonia (Clic here for more details about this article) |
7/26. 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 = 0.4keywords = pneumonia (Clic here for more details about this article) |
8/26. Intractable skin necrosis and interstitial pneumonia in amyopathic dermatomyositis, successfully treated with cyclosporin A.We report a patient with amyopathic dermatomyositis (DM) who mainly showed interstitial pneumonia and intractable skin necrosis in bilateral elbows and soles with a poor response to immunomediated therapy, including corticosteroid and high-dose intravenous immunoglobulin. Soon after starting oral cyclosporin A (CyA) the skin lesions healed completely and the interstitial pneumonia promptly improved in parallel with a decrease in serum KL-6. Since fatal interstitial pneumonia is frequently associated with amyopathic DM as in this case, administration of CyA should be actively considered as a therapeutic option when clinical symtoms are progressive and resistant to conventional treatments.- - - - - - - - - - ranking = 1.4keywords = pneumonia (Clic here for more details about this article) |
9/26. Three cases of 'mechanic's hands' associated with interstitial pneumonia: possible involvement with foot lesions.We describe three cases of the rare combination of lateral erythema and hyperkeratosis of the fingers that typify a condition known as 'Mechanic's Hands'. The first and the third cases were unusual in that the condition was associated with cutaneous involvement of the feet and interstitial pneumonia but not with myositis, or with only mild muscular involvement, while the second case was typical, being accompanied by myositis and detectable antibodies against histidyl transferase. We propose that Mechanic's Hands can occur in association with foot lesions and interstitial pneumonia, even if it is not accompanied by myositis.- - - - - - - - - - ranking = 1.2keywords = pneumonia (Clic here for more details about this article) |
10/26. Acute thrombotic thrombocytopenic purpura following doxycycline treatment of chlamydia pneumoniae infection in a patient with dermatomyositis.A 48-year-old white female who was suffering from dermatomyositis and chlamydia pneumoniae infection, developed acute rapidly fatal thrombotic thrombocytopenic purpura (TTP) following treatment with steroids and doxycycline. As a relationship between TTP and the inflammatory myopathies is now probably well established, it is very likely that our patient's TTP became manifest in association with dermatomyositis. Nevertheless, C. pneumoniae infection and doxycycline therapy cannot be excluded entirely as cofactors responsible for triggering her thrombotic microangiopathy.- - - - - - - - - - ranking = 1.2keywords = pneumonia (Clic here for more details about this article) |
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