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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical investigation for inflammatory myopathies, which include polymyositis (PM), dermatomyositis (DM) and others, was outlined. The serum creatine kinase (CK) activity increases in the majority of cases of inflammatory myopathies. However, the cases of myositis associated with connective tissue diseases tend to show normal or moderately elevated CK activity. Among the isoenzymes of CK, the MB fraction can increase in the course of treatment as it can originate from regenerating muscle fibres. The macro CK type 1 was reported to appear in association with myositides. Varieties of autoantibodies in the serum such as Jo-1 and Ku have been studied. The Jo-1 antibody is frequently detected in the cases of PM associated with interstitial pulmonary fibrosis. Examination of the heart and lungs is necessary, and so is a search for malignant neoplasms in the cases of DM. Muscle biopsy is mandatory for diagnosing PM, DM and other inflammatory myopathies. Among the latter, inclusion body myositis and granulomatous myopathy need to be identified before treatment as they generally respond poorly. Histological changes of inflammatory myopathies are often distributed unevenly. The magnetic resonance image and ultrasonography are helpful in estimating the distribution of the lesion and therefore in deciding the site of biopsy. Ultrastructural observation of the muscle showed invasion of activated lymphocytes under the basement membrane of the muscle fibres causing degeneration of the myofibrils. The subset analyses of infiltrating cells revealed considerable alterations after the steroid pulse therapy.
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PMID:[Clinical investigation for polymyositis and related disorders]. 747 50

Myositis linked to HTLV-1 is unfrequent. Over a period of 8 years, 14 patients with inflammatory myopathy were diagnosed in Martinique. Seven were seropositive for HTLV 1 antibody; the clinical and pathological data of whom are presented herein. Five patients presented with polymyositis, two with dermatomyositis. All seven patients had extra-muscular clinical features including neuropathy (4/7) and myelopathy (6/7), resulting in a quite peculiar clinical picture. Muscle biopsy showed a neurogenic process combined with myositic changes in 3/7 patients. Corticotherapy led to dramatic improvement in only one case, but with no sustained effect. HTLV 1 may be considered the etiological agent of this form of dermato-polymyositis, characterized by a clearly distinctive clinico-pathological picture, and a poor response to corticotherapy. As in the case of tropical spastic paraparesis/HTLV 1 associated myelopathy, careful assessment of non-steroidal therapy is now warranted.
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PMID:Clinical characteristics of HTLV-1 associated dermato-polymyositis. Seven cases from Martinique. 748 73

New information regarding myositis specific autoantibodies, histopathologic analysis of muscle biopsy specimens, and immunogenetic features of the different serologic subsets of disease has greatly increased our understanding of the pathogenesis of the inflammatory myopathies. The clinical descriptions of inclusion body myositis and 'amyopathic dermatomyositis' (Euwer and Sontheimer, 1993) are examples of our expanded descriptive capabilities in the evaluation of patients with myopathy. Finally, newer techniques such as cytokine analysis and magnetic resonance imaging may help in the ongoing assessment of disease activity in patients with myositis. The combination of these recently described clinical and laboratory parameters are enough to force a reconsideration of the previously described classification and diagnostic criteria in the inflammatory myopathies.
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PMID:Inflammatory myopathies. 749 35

We attempt to correlate the patient's disability and serum enzymes (creatinekinase, lactic dehydrogenase, aldolase, glutamic oxalacetic and glutamic piruvic transaminase) in several neuromuscular disorders using the Vignos and Archibald scale (V&A). In 806 cases we studied, serum enzyme levels and the V&A disability using a computer for Pearson's correlation and regressive analysis. A good correlation of the V&A with age suggested a progressive evolution (increased disability) in Duchenne muscular dystrophy, fascioscapulohumeral dystrophy, myotonic dystrophy, myopathies due to respiratory chain enzyme deficiency and amyotrophic lateral sclerosis. A negative correlation (decrease disability with age) was found for multicore myopathy, benign myopathy of childhood with type 1 predominance, carnitine myopathy deficiency and dermatomyositis. It was found a correlation (p < 0.05) of the V&A and the level of specific serum enzymes with Duchenne muscular dystrophy, oculocraniosomatic dystrophies, polymyositis and polyarteritis nodosa. Using regression analysis, we found a weak interrelation between serum enzymes studied simultaneously and the V&A. These weak relations suggest some limitation in the long term use of the five serum enzymes in the evaluation of neuromuscular disorders when compared with V&A; although they are very important in the diagnosis.
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PMID:[Correlation between functional disability, age, and serum enzymes in neuromuscular diseases]. 757 10

We describe a 41-year-old patient with adult-onset dermatomyositis who developed persistent pneumomediastinum and severe subcutaneous emphysema due to end-stage interstitial lung disease. The diagnosis of dermatomyositis was based on proximal muscle weakness, electromyographic findings of inflammatory myopathy, and positive findings on muscle biopsy. Low levels of creatine kinase elevation were found at the time of diagnosis (a form of dermatomyositis which has been associated with a poor prognosis). The patient had no signs of cutaneous vasculitis. Despite treatment with prednisone and azathioprine, she died of intercurrent gram-negative sepsis 15 months after the diagnosis of dermatomyositis.
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PMID:Chronic pneumomediastinum and subcutaneous emphysema: association with dermatomyositis. 771 58

A 74-year-old female patient with cutaneous ulcerations and typical dermatomyositis (DM) skin rash had no muscle disease for a 1-year and 5 months period. Histological examination of the skin ulceration indicated vascular occlusion without cellular infiltration. Cutaneous ulceration is a very rare manifestation of adult-onset DM patients without inflammatory myopathy.
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PMID:Cutaneous vasculitis in a patient with dermatomyositis without muscle involvement. 771 68

In this review, the main emphasis is on new advances concerning sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Polymyositis and dermatomyositis are reviewed briefly. Hypotheses are presented regarding the possible cause and significance of abnormally accumulated beta-amyloid protein, two other epitopes of beta-amyloid precursor protein, hyperphosphorylated tau, alpha 1-antichymotrypsin, ubiquitin, and prion protein in sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Because most of those proteins are also accumulated at the neuromuscular junction, "junctionalization" of other muscle fiber nuclei is a possibility. Attention is given to the fact that vacuolated muscle fibers in hereditary inclusion-body myopathy may represent early changes because they are virtually free of congophilic amyloid deposit but, like sporadic inclusion-body myositis, contain large accumulations of beta-amyloid protein and prion.
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PMID:Idiopathic inflammatory myopathies: inclusion-body myositis, polymyositis, and dermatomyositis. 780 66

In the last few years high-dose intravenous therapy with gammaglobulin in juvenile dermatomyositis with steroid resistant myopathy has been discussed as an alternative to cytostatic therapy. The 12-year-old Filipinogirl suffered from dermatomyositis with pronounced weakness of the limbs and masticatory muscles; creatinine-kinase was increased 22-fold. After 5 weeks of therapy with prednisolone (1.5 mg/kg body/weight/daily), the creatinine kinase decreased to sixfold of its standard value; the muscular weakness, however, progressed to almost complete paralysis of the scapular abductors and rotators and the coxofemoral flectors and rotators. Additional therapy with gammaglobulin at a dose of 1 g/kg body weight on two consecutive days at 4-week intervals led to complete restoration of muscular strength, normalization of muscle enzymes, and stabilization of these results after 12 courses of gammaglobulin and discontinuation of the steroid medication after 8 courses. This observation suggests that high-dose intravenous application of gammaglobulin represents an efficient therapy with minimal side effects in steroid-resistant juvenile dermatomyositis.
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PMID:[High dosage intravenous gamma globulin therapy in juvenile dermatomyositis]. 784 65

Myopathies produced by drugs are of vital concern and often confused with other more frequently diagnosed causes, such as the inflammatory myopathies of polymyositis/dermatomyositis and myositis secondary to toxic agents, metabolic and endocrine abnormalities, genetic predisposition, malignancies, and infections, particularly viruses. The drug-induced causes of myopathy warrant special emphasis because they are often overlooked, resulting in misdiagnosis and improper care.
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PMID:Drug-related myopathies. 785 22

Among the main concerns regarding the current therapy for the inflammatory myopathies are a lack of adequate controlled trials, a lack of objective means to reliably measure muscle strength, lack of natural history data, consideration of polymyositis, dermatomyositis, and inclusion-body myositis as a homogeneous group of inflammatory myopathies, and reliance on nonspecific markers for determining prognosis and assessing response to therapies. Prednisone remains the drug of choice in treating these disorders, although a controlled trial has never been undertaken to study its efficacy. Among the steroid-sparing agents, azathioprine, methotrexate, cyclosporine, and chlorambucil are used with invariably low to moderate success. There are no results of controlled trials to indicate whether one of these drugs is superior to another. Intravenous immunoglobulin, which is very expensive, was shown in a controlled trial to be effective in steroid-resistant dermatomyositis not only in dramatically improving muscle strength and skin rash but also in resolving the underlying immunopathology. Controlled trials of intravenous immunoglobulin in patients with polymyositis and inclusion-body myositis are under way. Inclusion-body myositis has emerged as a common inflammatory myopathy that is predictably disabling and resistant to most therapies.
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PMID:Current treatment of the inflammatory myopathies. 786 79


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