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1.
Celiac disease is usually associated with autoimmune disorders and has occasionally been reported in patients with inflammatory myopathies. Our aim was to determine the presence of celiac disease and antibodies associated with celiac disease in patients with inflammatory myopathies and to investigate their relationship. Serum antigliadin, anti-tissue transglutaminase, and antiendomysial antibodies were determined in 51 patients with inflammatory myopathies. HLA-DQ2 and -DQ8 alleles were studied to assess their complementary diagnostic value. Jejunal biopsy was performed in patients with moderate to high levels of antigliadin antibodies. Patients with jejunal histology consistent with celiac disease initiated a gluten-free diet. Seventeen patients (31%) were positive for antigliadin antibodies, which were significantly more frequent in patients with inclusion-body myositis than dermatomyositis (P < 0.001). Positive status to HLA-DQ2 and/or -DQ8 did not differ between antigliadin-positive (75% and 12.5%) or -negative (60% and 15%) patients. Three of five jejunal biopsies were diagnostic for celiac disease with histological normalization after a gluten-free diet. Thus, celiac disease is more prevalent in patients with inflammatory myopathies than in the general population. Positive status to HLA-DQ2 allele, which is known to be more frequent in patients with inflammatory myopathies, could explain the high prevalence of antigliadin antibodies in this population. The diagnostic value of HLA-DQ2 or -DQ8 haplotypes to detect celiac disease in patients with inflammatory myopathy is limited.  相似文献
2.
Zusammenfassung Nemaline-Strukturen bei einer progressiven Muskeldystrophie, einer Dermatomyositis und einer Neuromyopathie werden beschrieben. Es bestätigt sich die auf Grund von Einzelbeobachtungen geäußerte Auffassung, daß nemaline-Strukturen ein morphologisch unspezifisches Symptom darstellen. Nachdem sich auch das klinische Bild der nemaline-Myopathie mit zunehmenden Fall-beobachtungen bezüglich des Verlaufes, der Symptomatik und der Erstmanifestation als recht heterogen erwiesen hat, ist der Begriff nemaline-Myopathie als Krankheitseinheit unzureichen. Gegebenenfalls ist von gutartigen kongenitalen Myopathien mit nemaline-Strukturen zu sprechen.
The diagnostic specificity of nemaline-Structures
Summary Nemaline-structures seen in muscular dystrophy, dermatomyositis, and atypical neuromuscular disease with myxoviruslike inclusions are described. These observations confirm the interpretation that nemaline structures are an unspecific reaction arising in a heterogenic group of myopathies. Therefore, the appearance of nemaline structures doses not justify the assumption af a particular myopathic disease.
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3.
Summary Muscle biopsies from 12 patients with a typical clinical picture of dermatomyositis have been examined by electron microscopy. Endothelial cells of intramuscular blood vessels, their basal lamina, pericytes, muscle fibers, and satellite cells show degenerative or regenerative alterations. In nine patients, tubular arrays were noted in the cisterns of endoplasmic reticulum of endothelial cells, pericytes, lymphocytes, macrophages and satellite cells. Other types of inclusions were also observed. The pathogenesis of the disease is discussed.  相似文献
4.
Twelve patients with polymyositis or dermatomyositis were examined repeatedly during the course of the illness by muscle strength tests, serum enzyme determinations, frequency analysis of the electromyographic signal, and estimations of the spontaneous activity. All methods can be used to follow the course of the disease. Increasing muscle strength was correlated to decreasing serum enzyme values, decreasing spontaneous activity, and decreasing proportion of high-frequency components in the EMG signal, except during the initial period of treatment, when a temporary increase in high-frequency components was recorded.  相似文献
5.
One hundred and seven patients with polymyositis or dermatomyositis were followed for an average of 5 years. In 100 patients it was possible to evaluate the effect of treatment with steroids or a combination of steroids and immunosuppressive drugs. Fifty patients improved, 50 did not. Compared with the treatment-resistant group the treatment-responsive patients were of younger age, did not have an associated malignancy or cardiac disease and began treatment within 24 months after the onset of muscular weakness. In the treatment-responsive group the degree of improvement was correlated to the mean dose of steroids given during the first 3 months of treatment. Eighty-seven per cent of surviving responsive patients had no or slight disability at the end of the investigation. The mortality rate was 23% and was highest in the treatment-resistant group. Comparison between different treatment programs was possible and based on the results, a treatment schedule could be recommended. The principles of this schedule are: During the first month high doses of prednisone or prednisolone should be given daily. From the second month, an alternate-day administration can be employed. Tapering to maintenance dose should be gradual and slow. The treatment period should be long. The mean duration in the present series was 27 months.  相似文献
6.
Gastrointestinal investigation of 14 adult patients with polymyositis disclosed coeliac disease in five. The inflammatory myopathy in our patients is not the same as the myopathy often seen in coeliac disease with osteomalacia. One patient has been free from both gastrointestinal and muscular symptoms for 5 years on a gluten-free diet alone. The findings strongly suggest an association between polymyositis and adult coeliac disease.  相似文献
7.
Summary We present immunohistochemical, light-and electron-microscopic findings on a muscle biopsy specimen from a 21-year-old woman who developed debilitating dermatomyosisits in the course of toxoplasmosis. The muscle showed perifascicular muscle cell atrophy and prominent ultrastructural changes consistent with polymyositis. These myopathic changes were interpreted as an immunologic complication of systemic toxoplasmosis and were related to immunohistochemically demonstrable immune complex deposits in the small blood vessels. Our data suggest that dermatomyositis is caused and/or related toToxoplasma infection and is an immune complexmediated systemic disease.  相似文献
8.
The most typical clinical features of polymyositis (PM), the criteria of diagnosis and principles of treatment are outlined. An inflammatory disease of muscle, PM also frequently affects other organs such as the skin and hence the name dermatomyositis. The principal cardiac symptom is a peculiar disturbance of atrioventricular conduction, correlated with a specific anti-Ro autoantibody, present in 25% of patients. The etiology of PM is as yet unknown, although there is evidence for an autoimmune pathogenesis. It is frequently found in association with other immune-mediated diseases such as myasthenia gravis, pemphigus, immune-complex vasculitis and Sjogren syndrome. Laboratory investigations show hypergammaglobulinemia, a decrease of complement factors C3 and C4 and the presence of circulating immune complexes in 70% of patients. Very frequent, especially in cases of dermatomyositis, is a histologically detectable accumulation of IgG and complement in the walls of the intramuscular venous vessels. Cell-mediated hypersensitivity, emphasised formerly as highly significant in PM, has not been confirmed. The presence of specific antimyoglobin lymphocyto-toxicity, once considered to be the hallmark of muscle degeneration in PM, has been excluded by a number of laboratories. In a personal series of patients with various clinical forms of PM a severe loss of suppressor/cytotoxic lymphocytes was found in the peripheral blood and a relative increase in the first subset. These results support the hypothesis that a serious disturbance of immunoregulation is present in PM and is the cause of a multitude of immunological anomalies, the characterisation of which is under study.This work was supported by the Muscular Dystrophy Group of Great Britain  相似文献
9.
Summary Fourteen patients with polymyositis-dermatomyositis (PM-DM) underwent prospective cardiac assessment with non-invasive techniques. One patient had electrocardiographic evidence of Long-Ganong-Levine syndrome with multiple supraventricular premature beats. Echocardiographically one patient had late systolic prolapse of the posterior mitral leaflet and another had paradoxical movements of the interventricular septum. The study suggests that cardiac involvement is infrequent in PM-DM.  相似文献
10.
The infection of muscle is an infrequent condition. We report on a patient with a juvenile form of dermatomyositis who developed infectious myositis caused by Streptococcus pyogenes. The inflammatory myopathy probably favoured the colonization of muscle during a bacteremia related to the skin lesions. The main forms of streptococcal myositis, which can currently be differentiated by means of imaging techniques, are discussed in addition to its treatment and prognosis.  相似文献
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