1/102. subcutaneous emphysema with spontaneous pneumomediastinum and pneumothorax in adult dermatomyositis.We describe a 32-year-old patient with adult dermatomyositis who developed dyspnea and worsening of pre-existing infarcted skin lesions of the fingers. Chest radiographs showed diffuse hazy reticulonodular infiltration in both lungs, subcutaneous emphysema, pneumomediastinum, and pneumothorax. The pulmonary symptoms and cutaneous lesions gradually improved with a high dose of prednisolone. Although subcutaneous emphysema and pneumomediastinum occur frequently in association with traumatic disruption of cutaneous and mucosal barriers and assisted ventilation, it has rarely been observed in patients with interstitial pneumonitis in connective tissue diseases. Although dermatomyositis and subcutaneous emphysema are all relatively well-known diseases to dermatologists, the occurrence of spontaneous pneumomediastinum and pneumothorax and subsequent subcutaneous emphysema in connective tissue diseases such as dermatomyositis is unfamiliar. We discuss the possible mechanisms of this condition.- - - - - - - - - - ranking = 1keywords = interstitial pneumonitis, interstitial, pneumonitis (Clic here for more details about this article) |
2/102. 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.- - - - - - - - - - ranking = 6keywords = interstitial pneumonitis, interstitial, pneumonitis (Clic here for more details about this article) |
3/102. Myocardial fibrosis in polymyositis.Myocardial involvement in polymyositis is commonly suspected in noninvasive studies, but symptomatic cardiac disease is rare. We describe a 27-year-old woman with a 6 year history of severe polymyositis and persistent elevation of creatine phosphokinase-MB isoenzyme who suddenly developed congestive heart failure and bradycardia-tachycardia syndrome. autopsy revealed severe myocardial fibrosis without inflammatory cell infiltrates concomitant to active polymyositis of the skeletal muscles despite intensive longterm immunosuppressive therapy.- - - - - - - - - - ranking = 0.0016915138386807keywords = fibrosis (Clic here for more details about this article) |
4/102. dermatomyositis with elevated serum hyaluronate.We report two Japanese patients with dermatomyositis whose serum hyaluronate levels were correlated with their disease activities. The diagnosis of one patient was primary idiopathic dermatomyositis, and that of the second patient was dermatomyositis associated with neoplasia. The serum hyaluronate level of the former patient was decreased by systemic corticosteroid therapy, and that of the latter patient decreased following the surgical resection of mammary carcinoma and subsequent chemotherapy. The disease activity of each patient was greatly improved after these therapies. The longitudinal measurement of serum hyaluronate concentration may be useful to estimate the disease activity of patients with dermatomyositis whose initial serum levels of hyaluronate are elevated.- - - - - - - - - - ranking = 0.00036904237724896keywords = idiopathic (Clic here for more details about this article) |
5/102. Detection of anti-ADAM 10 antibody in serum of a patient with pulmonary fibrosis associated with dermatomyositis.OBJECTIVES: It has been suggested that the humoral immune system plays a part in the pathogenesis of pulmonary fibrosis. Although circulating autoantibodies to lung protein(s) have been suggested, few lung proteins have been characterised. The purpose of this study is to determine the antigen recognised by serum of a patient with pulmonary fibrosis associated with dermatomyositis. methods: To accomplish this, anti-small airway epithelial cell (SAEC) antibody in a patient's serum was evaluated using a western immunoblot. RESULTS: An autoantibody against SAEC was found, and the antigen had a molecular weight of 62 kDa. Using the patient's serum, clones from the normal lung cDNA library were screened and demonstrated that anti-SAEC antibody in the patient's serum was against ADAM (A disintegrin and metalloprotease) 10. CONCLUSION: This is the first report that demonstrates the existence of anti-ADAM 10 antibody in a patient with pulmonary fibrosis associated with dermatomyositis.- - - - - - - - - - ranking = 0.66381336429513keywords = pulmonary fibrosis, fibrosis (Clic here for more details about this article) |
6/102. dermatomyositis associated with malignancy. 12 case reports.dermatomyositis (DM) is an idiopathic inflammatory disease of unknown etiology which manifests itself with symmetrical proximal muscle weakness and typical heliotrope skin rash. Internal malignancy is a specific trigger for some cases of DM. The aim of this study is to find predictive signs of cancer in dermatomyositis patients. Twelve (2 males and 10 females) (24%) out of 50 adult patients with DM (10 males and 40 females between 21 and 81 years old) had associated with malignancy (paraneopastic dermatomyositis, PDM). The male: female ratio in patients with idiopathic dermatomyositis (IDM) was 1:3.75 and for PDM it was 1:5. The frequency of dermatomyositis increases with age. PDM is most frequent between 40 and 69 years of life. Associated malignancies were respiratory tract carcinomas in males, genital and breast carcinomas in females. DM preceded the carcinoma in 5 cases (41.7%); 7 patients (58.3%) had already been treated for malignancy. In conclusion PDM in our country is more frequent in females and in the age after 40 years. association of dermatomyositis with malignancy is relatively high.- - - - - - - - - - ranking = 0.00073808475449792keywords = idiopathic (Clic here for more details about this article) |
7/102. 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 = 0.038307212364923keywords = interstitial (Clic here for more details about this article) |
8/102. Pneumomediastinum in dermatomyositis: association with cutaneous vasculopathy.OBJECTIVES: To study the pathogenesis of pneumomediastinum in polymyositis/dermatomyositis (PM/DM). patients AND methods: The clinical records of 48 patients with PM/DM were reviewed, focusing mainly on the presence of pneumomediastinum and cutaneous vasculopathy, and the chest radiographic changes. A patient with pneumomediastinum with a characteristic change in his bronchus is described in detail. case reports of pneumomediastinum in PM/DM in English publications are reviewed. RESULTS: Among the 48 patients with PM/DM, pneumomediastinum was observed as a complication in four patients with DM and none of the patients with PM. Three of the four patients with pneumomediastinum, but only six of the 44 patients without this complication, had associated cutaneous vasculopathy. There was a significant association of pneumomediastinum with cutaneous vasculopathy (p = 0.02) and younger age (p = 0.04), but not with the prevalence of lung disease. A 30 year old man (patient 1) with DM, who had interstitial pneumonitis and skin ulceration due to vasculopathy, developed pneumomediastinum. Fibreoptic bronchoscopy showed white plaques on the bronchial mucosa, which were confirmed by microscopic examination as representing subepithelial necrosis. A literature review showed 13 cases of DM but no patient with PM with pneumomediastinum. CONCLUSIONS: In patient 1, bronchial necrosis due to vasculopathy was strongly suspected as being responsible for the pneumomediastinum. The results suggest that pneumomediastinum was associated not with interstitial pneumonitis but with the complication of vasculopathy appearing as skin lesions in DM.- - - - - - - - - - ranking = 2keywords = interstitial pneumonitis, interstitial, pneumonitis (Clic here for more details about this article) |
9/102. 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 = 0.22984327418954keywords = interstitial (Clic here for more details about this article) |
10/102. A causal role for parvovirus B19 infection in adult dermatomyositis and other autoimmune syndromes.BACKGROUND: infection with parvovirus B19 (B19) has been associated with connective tissue disease (CTD) stigmata, namely, a systemic lupus erythematosus (SLE)-like illness, seronegative polyarthritis resembling rheumatoid arthritis, and vasculitis. The dermatopathology and pathogenetic basis of such B19-associated CTD-like syndromes have not been elucidated. OBJECTIVE: We attempted to document persistence of the B19 genome in skin lesions of 7 patients with CTD-like symptomatology following B19 infection and to correlate systemic manifestations to dermatopathological findings. METHOD: In 7 prospectively encountered patients in whom history, clinical signs and/or serology supported a diagnosis of CTD in the setting of B19 infection, dermatopathological and clinical features were correlated. parvovirus B19 viral genome was sought in skin tissue using the polymerase chain reaction (PCR). RESULTS: Two patients had clinical features diagnostic of myopathic dermatomyositis (DM), 1 of whom is still symptomatic 1.5 years after the onset of her illness, and the other has had typical clinical features of DM for a duration of 3.5 years. A 3rd patient with SLE remains symptomatic 4 years after the onset of her illness. A 4th patient has persistent seronegative symmetrical polyarthritis of 6 years' duration and cutaneous lesions of granuloma annulare (GA). The 5th patient has a 1.5-year history of debilitating polyarthritis and cutaneous lesions with overlap features of DM and subacute cutaneous LE (SCLE). The 6th patient has had a persistent folliculocentric necrotizing vasculitis for 3 years. The 7th patient has a 1-year history of microscopic polyarteritis nodosa (PAN) with cutaneous vasculitis and persistent active renal disease. In 4 patients, exposure to children with fifth disease immediately preceded the onset of their CTD. parvovirus B19 infection was documented serologically in 6 patients with antibodies of IgG subclass in 6 and of IgM subclass in 1. Four of 6 patients questioned had a history of atopy. skin biopsies from patients with clinical features of SLE or DM demonstrated an interface dermatitis with dermal mucinosis. A necrotizing vasculitis with epithelial pustulation was seen in 2 patients. Interstitial GA-like infiltrates were seen in 5 cases. Immunofluorescent (IF) testing revealed a positive lupus band test (LBT) and epidermal nuclear and vascular staining for IgG and C5b-9 in the SLE patient. One DM patient had a negative LBT in concert with C5b-9 deposition along the dermoepidermal junction (DEJ) and within blood vessels while the other showed endomysial vascular Cs5b-9 deposition. In all patients, skin biopsy material contained B19 genome, which was absent in the serum of 4 patients analyzed. Symptomatic relief followed immunosuppressive and immunomodulatory therapy with agents including prednisone, cyclophosphamide, hydroxychloroquine, non-steroidal anti-inflammatory drugs and etanercept, but no patient has had complete symptom resolution. CONCLUSIONS: Persistent B19 infection may be of pathogenetic importance in certain prototypic CTD syndromes, to which underlying immune dysregulation associated with a blunted IgM response to viral antigen may predispose. Anti-viral therapy might be worthy of consideration since traditional immunosuppressive therapy was unsuccessful in our cases.- - - - - - - - - - ranking = 5.0459808781001E-5keywords = exposure (Clic here for more details about this article) |
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