Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fas/Fas ligand (FasL) interaction can induce apoptosis, have a costimulatory role or act as a mechanism by which cytotoxic T cells produce target cell lysis. We used several commercially available antibodies to study Fas and FasL expression in polymyositis (PM), inclusion body myositis (IBM), dermatomyositis (DM) and normal controls. A strong Fas signal occurred on the sarcolemma, and to a lesser extent in the sarcoplasm of neural cell adhesion molecule (NCAM)-positive or developmental myosin heavy chain-positive regenerating muscle fibers and of injured fibers with presumed abortive regenerative activity, including some nonnecrotic invaded fibers in PM and IBM and some of the atrophic perifascicular fibers in DM. Most fibers within groups of atrophic fibers in IBM were strongly Fas-positive, and statistically more muscle fibers were Fas-positive in IBM compared to PM. A subset of the actively invading CD8+ T cells in nonnecrotic muscle fibers in PM and IBM, and scattered CD4+ cells in each inflammatory myopathy, had up-regulated Fas expression, probably reflecting costimulation. No FasL antibody consistently labeled the positive control tissue (testis) or intramuscular elements in control or inflammatory myopathy specimens. Our study identifies regenerating muscle fibers as the main site of Fas immunoreactivity in inflammatory myopathies, and Fas expression may be part of an activated or reactivated developmental program of new gene expression in regenerating or denervated muscle fibers. Our data plead against a specific role of Fas/FasL interaction in the immunopathogenesis of the inflammatory myopathies.
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PMID:Immunolocalization of FAS and FAS ligand in inflammatory myopathies. 1151 85

Dermatomyositis and polymyositis are treatable disorders of skeletal muscle. Despite their clinical similarities, they appear to have fundamentally different autoimmune origins. Inclusion body myositis, from its origins 30 years ago, has emerged as the commonest acquired myopathy of the elderly. Despite inflammatory changes, it is unclear whether it should be considered a primary inflammatory myopathy, and it generally responds poorly to the same treatments that are effective in other inflammatory myopathies.
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PMID:Inflammatory muscle diseases. 1156 70

The idiopathic inflammatory myopathies (IIM), including dermatomyositis (DM), polymyosititis (PM) and inclusion body myositis (IBM), are a group of autoimmune diseases characterized by chronic lymphocytic and macrophagic infiltration in muscle. The mechanism for recruitment of these cells probably involves chemokines. We have previously reported that monocyte chemoattractant protein-1 (MCP-1), a beta chemokine, seems to play a major role in mononuclear cell recruitment especially in DM. Here we have investigated the distribution of the main MCP-1 receptors CCR2A and CCR2B in IIM by polymerase chain reaction (PCR), immunohistochemistry and in situ hybridization. We have shown by reverse transcription-PCR that both CCR2A and CCR2B were expressed at low level in normal muscle and that CCR2A was up-regulated in IIM (P=0.02) and was higher in PM and IBM than in DM (P=0.04). By immunohistochemistry and in situ hybridization we have observed that CCR2 isoforms were expressed by different cell subsets in both normal and IIM muscle. CCR2A was expressed in vessel walls and by some mononuclear cells, especially in cells involved in partial invasion in PM and IBM. CCR2B expression was observed in all satellite cells, in the muscular domain of neuromuscular junctions and in some regenerative fibers of IIM, but not in inflammatory exudates. In conclusion, the present study highlights the major role played by MCP-1 and its counter-receptor CCR2 in the pathophysiology of IIM, and shows that the CCR2 receptors are cell specific. The variation of the total amount of CCR2A and its local distribution according to the type of IIM might be a new path towards the understanding of the constitution of mononuclear infiltrates in IIM.
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PMID:CCR2A and CCR2B, the two isoforms of the monocyte chemoattractant protein-1 receptor are up-regulated and expressed by different cell subsets in idiopathic inflammatory myopathies. 1160 15

The inflammatory muscle diseases dermatomyositis, polymyositis and inclusion body myositis are of unknown cause, but immune mechanisms are strongly implicated. Progress in the past two years has led to an improved understanding of the main molecular events involved in the immunological synapse between muscle and autoinvasive T cells. In particular, we now have a better understanding of TCR gene rearrangement in endomysial T cells, regulation of MHC expression, activity of co-stimulatory molecules, and the signalling cascades activated by cytokines, chemokines and metalloproteinases. Recent reports of an upregulation of strong anti-apoptotic molecules on the surface of muscle fibers identifies the end result of these disease processes, loss of muscle cells, as through necrosis, and not apoptosis. Such progress in molecular immunopathology has generated the interest to apply semispecific immunotherapies with the hope of halting disease progression or improving the strength of patients unresponsive to currently available non-specific immunotherapeutic interventions.
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PMID:The molecular and cellular pathology of inflammatory muscle diseases. 1171 55

Hypertrophy of the cricopharyngeal muscle is a serious clinical condition that can cause severe dysphagic symptoms, including prolonged deglutition and postdeglutitive aspiration. Although the therapeutical concepts are well established, the pathogenic mechanism of cricopharyngeal hypertrophy remains unclear. We present a patient with a ten-year history of progressive dysphagia. The neurological and MRI findings were normal. However, videocineradiography showed severe hypertrophy of the cricopharyngeal muscle. This condition was first treated by injections of botulinum toxin, which did not alleviate the symptoms. Next, myotomy and muscle biopsy were performed. Histological evaluation disclosed lymphoplasmacellular florid myositis, single-fiber atrophy, and muscle fiber necrosis with phagocytosis. There were no signs of inclusion body myositis or oculopharyngeal muscular dystrophy. Our finding of severe cricopharyngeal muscle hypertrophy associated with myositis has been published previously (n = 34). The study presented here shows cricopharyngeal dysphagia associated with various systemic diseases, including motor neuron disease, general granulomatous disease, dermatomyositis, or inclusion body myositis. Isolated changes of the cricopharyngeal muscle were described in 65% of the cases.
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PMID:Cricopharyngeal muscle hypertrophy associated with florid myositis. 1172 Mar 99

Reactive oxygen intermediates (ROI) and nitric oxide (NO(.)) are produced in abundance in the inflammatory muscle diseases of autoimmune origin polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM). However, their role in the pathogenesis of these diseases is so far not clear. In contrast to demyelinating neuropathies, there is no convincing evidence for oxide-induced apoptosis either in myocytes or in lymphocytes and phagocytes in inflammatory myopathies. On the contrary, NO(.) released at low concentrations at target sites may even have cell-protective effects. A major mechanism of protection from apoptosis in both myocytes and inflammatory cells seems to be the upregulation of anti-apoptotic proteins like Bcl-2. Caution is warranted to apply antioxidative and anti-apoptotic agents to patients with inflammatory myopathies as long as the pathogenic role of oxides and apoptosis in the individual case is not resolved.
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PMID:Oxides and apoptosis in inflammatory myopathies. 1174 63

Standard drug therapy of adult polymyositis, dermatomyositis and inclusion body myositis includes high-dose corticosteroids and cytotoxic drugs (methotrexate, azathioprine (AZA) and cyclophosphamide). Recent data are in favour of the early introduction of a cytotoxic or immunomodulating drug in addition to corticosteroid therapy. In patients with corticosteroid- and cytotoxic-resistant myositis, promising novel approaches to management include: iv. megadose pulse methylprednisolone combined with cytotoxic drugs, combination therapy with both methotrexate and AZA, cyclosporin, tacrolimus, fludarabine and iv. immunoglobulin (IVIG). Recent advances in the understanding of the role of cytokines and complement, in the pathogenesis of myositis, have led to preliminary therapeutic trials of three biological agents: etanercept, infliximab and anti-C5 monoclonal antibody.
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PMID:Recent advances in the management of adult myositis. 1177 50

The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic diseases that include the familiar disease entities of dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM). A subset of patients has unique autoantibodies which are specific for IIM (myositis specific autoantibodies; MSAs). We studied the clinical and serological characteristics of IIM in 125 Dutch patients. Sera were analysed by immunoblotting, enzyme-linked immunosorbent assay, and immunoprecipitation. The most frequently encountered MSA was the anti-Jo-1 autoantibody (20%), followed by anti-tRNAHis (6%), anti-Mi-2 (6%), and anti-SRP (4%). The presence of certain MSAs was clearly associated with specific clinical characteristics. Anti-Jo-1 and anti-tRNAHis were associated with the anti-synthetase syndrome, anti-SRP with PM with severe myalgia and arthralgia and a moderate response to immunosuppressive treatment. A novel finding was the presence of anti-Mi-2, not only in DM, but also in PM. MSAs were frequently present in DM/PM sera, but were hardly ever detected in the sera of IBM patients. The few IBM patients with MSAs demonstrated a significant response to immunosuppressive treatment. It can be concluded that MSAs define specific clinical syndromes within the spectrum of IIM and that they can assist in the differential diagnosis and treatment plan of these enigmatic disorders by virtually excluding IBM by their presence, and by potentially identifying a subgroup of steroid-responsive IBM patients.
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PMID:Clinical and serological characteristics of 125 Dutch myositis patients. Myositis specific autoantibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies. 1195 71

Inflammatory muscular diseases of adult and child consist of dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM). Muscular biopsy takes a seminal place in their diagnosis, through analysis of the topography and clustering of individual histological lesions: endomysial, perimysial and perivascular inflammation, muscular necrosis with regeneration, fibre modifications, fibrosis, micro-angiopathy. They can be associated with collagen diseases or malignant tumors that usually precede them. IBM seems somewhat apart among inflammatory myopathies, being characterised by the association of neurogenic and myogenic features and the presence of vacuoles containing filaments with an accumulation of proteins previously reported in Alzheimer's disease (beta amyloid protein, tau, ubiquitin,.). Inflammation is of various intensity, lacking in familial IBM (hereditary inclusion body myopathy) that otherwise shares the same histologic characteristics as sporadic forms. Other inflammatory muscular diseases: focal myositis, eosinophilic polymyositis, are less frequent. Macrophagic myofasciitis, viral myositis and drug induced myositis are discussed in other articles.
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PMID:[Histological data in inflammatory myositis]. 1196 88

The group of idiopathic inflammatory myopathies encompasses polymyositis, dermatomyositis and inclusion body myositis. These diseases share the following features: progressive muscle weakness, an increase in serum creatine kinase activity and the presence of mononuclear cell infiltrates in the muscle biopsy. Polymyositis, dermatomyositis and inclusion body myositis are differentiated on the basis of the distribution of muscle weakness, and specific histopathological features. Many specialties may see these patients as the clinical presentation can vary widely and may be atypical, requiring further diagnostic procedures. A 40-year-old man with a heliotrope rash and periorbital oedema, but no muscle involvement, was diagnosed with dermatomyositis sine myositis. He was successfully treated with corticosteroids but died later of cardiac failure. A 72-year-old man with a pulmonary malignancy subsequently developed the clinical features of dermatomyositis. Steroid therapy diminished the complaints but he died of pulmonary embolism. A 54-year-old woman with the clinical features of inclusion body myositis did not have rimmed vacuoles in her muscle biopsy specimen and was initially erroneously diagnosed with polymyositis, for which she was treated with corticosteroids, but without beneficial effect.
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PMID:[Three patients with divergent presentations of idiopathic inflammatory myopathy]. 1203 18


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