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

The localization of focal inflammatory myopathy (IM) is aided by MRI, which facilitates diagnostic testing (muscle biopsy or EMG). Antibodies to antigens involved in protein synthesis are specific for IM and characterize distinct subsets of adult IM, but are rare in pediatric IM. In definite juvenile dermatomyositis, serological indicators of disease activity include neopterin and von Willebrand factor antigen, markers of macrophage activation, and endothelial cell damage, respectively; nailfold capillarioscopy documents small blood vessel involvement. Monitoring the percentage of circulating B cells may indicate the response to therapy.
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PMID:Juvenile dermatomyositis (JDMS): new clues to diagnosis and pathogenesis. 795 31

The immunopathogenic mechanisms responsible for myasthenia gravis, polymyositis, masticatory myopathy, and dermatomyositis are discussed in light of their relevance to the clinical disease. Current thinking concerning these disorders is presented as a prelude to further research and greater understanding.
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PMID:Immune-mediated muscle disease. 797 43

Prognostic factors were analysed in 77 patients with idiopathic inflammatory myopathy identified over a 5 year period. Formal statistical tests did not differentiate useful prognostic indices and a polymyositis disability score was devised in an attempt to gain some prognostic information. Partial (47%) or full (31%) recovery were seen in most cases with no recovery of strength (9%) and death (11%) being less common outcomes. Onset before the age of 50 and duration of symptoms of less than 12 months prior to presentation were favourable prognostic features, and treatment with regimes other than steroid therapy alone a probable favourable indicator. Level of creatine kinase (CK) at presentation and histopathological separation of dermatomyositis or polymyositis failed to alter prognosis. Most patients who died did so within the initial 6 months of treatment. Four of five patients < 50 years old with either a fatal outcome or no improvement were treated with steroids alone. Large multi-centre studies are required to provide reliable data about prognostic factors in idiopathic inflammatory myopathies and the methodology used in this study can only be regarded as providing pointers towards possible prognostic factors rather than being definitive.
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PMID:Biopsy proven polymyositis in Victoria 1982-1987: analysis of prognostic factors. 804 1

Dermatomyositis (DM) is characterized by an inflammatory myopathy and typical cutaneous lesions. In a retrospective study, 29 adult cases without overlap syndrome were reviewed. Our aim was to define predictive signs of cancer. Ten (four women, six men) of the 29 patients (35%) had DM associated with malignancy. The diagnosis of cancer was made concomitantly in six cases, more than year after (two cases), more than year prior to (two cases) the occurrence of DM. No extensive diagnosis procedures were necessary for identifying the tumors. A paraneoplasic evolution was certain for any patient, probable for six, improbable for fourth. For the group with malignancy: age was higher (P = 0.01), albumin lower (P = 0.04), C reactive protein higher (P = 0.0061). These parameters seem to have predictive value for the presence of malignancy.
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PMID:[Predictive signs of cancers in dermatomyositis. Study of 29 cases]. 805 49

Although there have been considerable advances in our understanding of the immunopathogenesis of the different forms of autoimmune inflammatory myopathy, the treatment of these conditions remains largely empirical, being based upon the use of immunosuppressive and immunomodulatory therapies which, for the most part, are non-selective in their actions. Corticosteroids are usually effective in adult and childhood cases of polymyositis and dermatomyositis, but are only rarely helpful in inclusion body myositis, which is usually also unresponsive to other forms of immunosuppressive therapy. Alternate-day corticosteroid therapy has a role in patients with mild disease and as a means of minimizing the side-effects of steroids. This may also be achieved by the early introduction of a second-line agent such as methotrexate or azathioprine, which will allow more rapid steroid withdrawal and may also improve the chances of inducing a remission in more severe cases. In patients who fail to respond adequately to oral corticosteroids, or who relapse after an initial response, intravenous immunoglobulin therapy or pulse therapy with intravenous methylprednisolone are promising approaches which appeal as safer alternatives to cytotoxic drugs. However these forms of treatment will require further evaluation in prospective clinical trials. The same applies to cyclosporin, which has a more selective action on T cells, and which has been reported to be effective in resistant cases of adult and juvenile polymyositis and dermatomyositis. In the longer term, the development of more specific forms of immunotherapy for these myopathies, aimed at blocking autoantigen presentation or its interaction with T cells, awaits the identification of the target antigens and T cells which initiate the autoimmune process.
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PMID:Treatment of inflammatory myopathies. 815 49

The purpose of this report is to describe our initial experience with techniques employing magnetic resonance imaging (MRI) to guide the choice of muscle to be biopsied in patients suspected of having inflammatory myopathy. Five patients with a clinical diagnosis of inflammatory myopathy (IM) were studied. Four were imaged prior to biopsy. Four had repeated examinations, either immediately following biopsy or to evaluate disease progression. Use of MRI to localize muscle lesions was associated with abnormal pathologic findings in all cases, including histopathologic demonstration of lymphocyte infiltration in three cases of idiopathic polymyositis; nonspecific myopathic changes were seen in one patient with probable dermatomyositis and in one patient with chronic inflammatory polyneuropathy and high serum creatine kinase levels (> 45,000 IU/ml). The precise location of the area sampled by biopsy was visible in only one of four postbiopsy images. MRI shows promise in identifying pathologic muscle in patients suspected of having one of the inflammatory myopathies; however, further refinement of localization techniques may be needed to optimize histopathologic diagnoses.
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PMID:MRI-guided biopsy in inflammatory myopathy: initial results. 827 95

Dermatomyositis is a disease of unknown cause, characterized by a diffuse inflammatory myopathy and skin changes consisting of heliotrope eruption, Gottron's papules, periungual telangiectasia with dystrophic cuticles, skin telangiectasia, and atrophy. Ocular involvement may consist of conjunctival edema, nystagmus, extraocular muscle imbalance (when associated with myasthenia gravis), iritis, cotton-wool spots, and optic atrophy. Conjunctival avascularity and membranous conjunctivitis also have been reported. We describe an unusual conjunctival finding with secondary corneal surface disturbances in a patient with dermatomyositis.
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PMID:Exuberant conjunctival pseudopolyposis in a patient with dermatomyositis. 829 66

Molecular hybridization using an enterovirus group specific probe detected virus RNA in muscle biopsy samples from 25 of 96 cases of inflammatory muscle disease and similarly from 41 of 158 cases of postviral fatigue syndrome (PFS). Enterovirus RNA was detected in only two of 152 samples of control muscle. The inflammatory myopathy group comprised patients with polymyositis (PM), juvenile dermatomyositis (JDM) or adult dermatomyositis (DM), and all showed the presence of an inflammatory infiltrate and fiber necrosis on histological examination of a muscle biopsy sample. In contrast, muscle samples from the PFS group were histologically normal except for non-specific changes such as occasional single fiber atrophy. By analogy with enteroviral myocarditis, which can progress to a post-inflammatory disease with persistence of virus in myocardium and disposes to the rapid development of dilated cardiomyopathy, we propose that PFS syndrome may be a sequela of a previous inflammatory viral myopathy.
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PMID:Persistence of enterovirus RNA in muscle biopsy samples suggests that some cases of chronic fatigue syndrome result from a previous, inflammatory viral myopathy. 840 78

We investigated the profiles of cytokine mRNA expression in muscle in 15 cases of inflammatory myopathy (IM) (5 each of polymyositis, inclusion body myositis, and dermatomyositis) and in 10 controls (5 of Duchenne dystrophy and 5 non-weak subjects). Expressions of the predominantly T cell-derived cytokines (interleukin (IL)-2, IL-4, IL-5, and interferon-gamma (IFN-gamma), of the predominantly macrophage-derived cytokines (IL-1, IL-6, and tumor necrosis factor-alpha (TNF-alpha)), as well as cytokines that can be of either T cell or macrophage origin (granulocyte-macrophage colony stimulating factor (GM-CSF) and transforming growth factor beta 1 (TGF-beta 1) and TGF-beta 2), were monitored by the reverse transcriptase-PCR method. The expression of T cell cytokine mRNAs for IL-2, IL-5, and IFN-gamma was generally weak or inconsistent. IL-4 mRNA expression was consistently moderate to strong in polymyositis but generally weak or absent in the other IMs. The expression of macrophage cytokine mRNAs for IL-1 alpha and IL-1 beta was weak or absent in all cases. Variable TNF-alpha mRNA expression was observed in 12 of 15 IM cases and faint or weak expression in 5 of 10 controls. Very strong GM-CSF expression was detected, but only on boosted PCR, in 12 of 15 cases of IM but in none of the controls. IL-6 was expressed only weakly or inconsistently. In contrast to the variable expression of several of the above mentioned cytokine mRNAs, all IM specimens strongly expressed TGF-beta 1 mRNA and 12 of 15 strongly expressed TGF-beta 2 mRNA. Thus, with the exception of IL-4 expression in polymyositis, a similar pattern of cytokine mRNA expression exists in the different types of IMs. Moreover, this pattern resembles that detected in non-weak and DD controls, although expression is generally weaker in the non-weak controls. The findings suggest that in IM muscle a sustained secretion of cytokines by T cells or of IL-1 by macrophages is not a prerequisite for operation of the immune effector response and that muscle may not be the site of ongoing sensitization.
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PMID:Analysis of cytokine expression in muscle in inflammatory myopathies, Duchenne dystrophy, and non-weak controls. 855 29

Polymyositis/dermatomyositis are rare autoimmune diseases. Classification is usually performed according to the criteria of Bohan and Peter. The occurrence of myositis-specific autoantibodies has recently been described in inflammatory myopathies. Approximately half of the patients can now be classified by these specific autoantibodies. Several of these autoantibodies (anti-aminoacyl-tRNA synthetases, anti-SRP, anti-Mi2) are strongly associated with the clinical presentation. We may expect that in the future different subsets of these diseases will be increasingly identified by serum antibodies. We report on a patient with myopathy, pulmonary fibrosis and polysynovitis, a typical clinical presentation of the anti-Jo1 syndrome (anti-synthetase syndrome).
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PMID:[Myositis, polysynovitis and pulmonary fibrosis: anti-Jo-1 syndrome]. 857 93


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