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

Forty-one children with dermatomyositis who were treated with corticosteroids and who have been followed over a period of 15 years have been presented. Progressive proximal muscle weakness was seen in all and 60% had muscle pain. The skin rash considered classic for dermatomyositis was seen in 33 children at the time of diagnosis. Elevation of serum muscle enzymes, electromyographic abnormalities, and muscle biopsy evidence of acute myositis were of confirmatory diagnostic value. The course of the disease in this study group has reconfirmed the efficacy of adrenal corticosteroid treatment in conjunction with an individualized physical therapy program and consistent followup. Prognosis for life and minimal functional disability has been good. There have been 3 deaths recorded in this series, only one of which was certain in its relationship to dermatomyositis.
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PMID:Dermatomyositis in the pediatric patient. 26 8

Previous nuclear disease was found twice: Mc Ardle disease, dermatomyositis. Causative factors were: strenous exercise, hyperthermia, intoxication, influenza. Myalgias and/or myoedema was recorded in ten cases, associated with an hypovolemia of variable severity in eight. Oligo-anuria was observed in eight cases. The acute renal failure (ARF) was characterized by an increase in the serum creatinin more important than the rise in the blood urea and, in some cases, severe metabolic disturbances: hyperkaliema (6 cases), hypocalcemia (5 cases), hyperphosphatemia (5 cases) and hyperuricemia (5 cases). Diagnosis was made by the increase in sera of the muscles enzymes, specially the CPK and the search for myoglobinuria, positive during the first seven days. A complete recovery of renal function was observed in the nine survivors with a transient and moderate hypercalcemia in three. Three patients had persistant neuro-muscular deficiencies. Non traumatic rhabdomyolysis is not a rare cause of ARF and should be considered when the etiology of ARF is uncertain.
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PMID:[Acute renal failure due to non traumatic rhabdomyolysis. 11 cases (author's transl)]. 53 Sep 46

Animal models have proven very useful in furthering insight into a number of muscle diseases. Studies of ethanol-fed rats are being used to understand the pathogenetic mechanisms underlying acute and chronic myopathy induced by ethanol. Several animal species, including mice, dogs, and cats, develop X-linked muscular dystrophies, which have genetic defects identical to those of Duchenne muscular dystrophy. As in the human disease, these animals lack dystrophin. They are being used to investigate the mechanisms by which lack of dystrophin results in weakness and to examine myoblast transfer as a treatment modality. A model of eosinophilia-myalgia syndrome has recently been induced in Lewis rats by the feeding of L-tryptophan samples that were implicated in the clinical syndrome in humans, making possible studies of the pathogenesis of this interesting new entity. A dermatomyositis-like syndrome occurs spontaneously in dogs, and polymyositis-like illnesses can be induced in mice by immunization with muscle or following infection with selected viruses, especially enteroviruses. Study of the latter is helping us understand mechanisms in the etiology and pathogenesis of inflammatory myositis and virus-induced autoimmunity.
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PMID:Animal models of myopathy. 177 47

Specimens of muscle and fascia from 13 patients fulfilling the Centers for Disease Control criteria for the eosinophilia myalgia syndrome (EMS) were studied by quantitative immunocytochemical analysis. The immunolocalization of CD3, CD4, CD8, CD22, and CD56 markers, the gamma delta T-cell receptor, major histocompatibility complex (MHC) class I complex and class II antigens, and complement membrane attack complex (MAC) were examined. The distribution and relative proportions of T cells and T-cell subsets, B cells, macrophages, and eosinophils were determined at perivascular, perimysial, endomysial, and fascial sites of accumulation. At all sites, T cells were predominant, CD8+ cells outnumbered CD4+ cells 6- to 20-fold, and between 60 and 80% of T cells were activated. B cells and eosinophils each accounted for less than 3% of inflammatory cells. Very few cells expressed either the gamma delta T-cell receptor or natural killer cell markers. As in dermatomyositis (DM), MHC class I antigen complex expression was increased on many structurally normal muscle fibers, but in contrast to DM, microvascular MAC deposits were not a feature of EMS. The findings implicate a cellular immune response directed against a connective tissue component in EMS.
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PMID:Eosinophilia myalgia syndrome: I. Immunocytochemical evidence for a T-cell-mediated immune effector response. 185 82

This previously healthy 43-year-old man was admitted to our hospital with a history of rash, dysphagia and severe myalgia for two months. Physical examination showed prominent edema and erythema over the face and the chest, scattered ulcerations on the trunk, and muscle atrophy most prominent proximally. Serum levels of muscle enzymes were remarkably increased. Two weeks of oral prednisolone therapy (40 mg/day) was not effective, and betamethasone intravenous pulse therapy (3 x 1000 mg) was followed by slight clinical improvement. However, 12 days after pulse therapy, he complained abdominal pain on the right lower quadrant. The surgical findings included peritonitis due to single perforation of the cecum. After operation, cyclosporine therapy was added and over the next 14 month a considerable clinical improvement was noted. Prednisolone was reduced from 80 mg to 10 mg daily. Biopsy specimens from ulcerated+ skin and perforated cecum showed prominent vascular abnormalities: arterial and venous intimal hyperplasia, occlusion of vessels by fibrin thrombi, and lymphocytic infiltration which affected veins of all sizes. The evidence strongly suggests that both skin ulcers and cecum perforation were caused by vasculitis and occlusion of vessels, which often seen in childhood dermatomyositis.
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PMID:[Adult dermatomyositis with angiopathy and cecum perforation]. 188 78

Fourteen patients with childhood scleromyositis followed from 1 to more than 10 years experienced concomitant sclerodermoid and dermatomyositis features, variably expressed at one time or another during the course of the disease. The most characteristic features were myalgia-myositis, arthralgia-arthritis, puffy, atrophic, sclerotic fingers, and Raynaud's phenomenon. This overlap syndrome was the most frequent sclerodermoid condition in children, differing from both systemic scleroderma and dermatomyositis. The course of the disease was protracted and rather benign, and PM-Scl antibody was an important diagnostic and prognostic marker. We present criteria for diagnosis of scleromyositis and its differentiation from systemic scleroderma, dermatomyositis, and Sharp overlap syndrome.
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PMID:Childhood scleromyositis: an overlap syndrome associated with PM-Scl antibody. 190 68

The recent delineation of a clinical syndrome marked by eosinophilia, myalgia, and scleroderma-like skin changes associated with L-tryptophan use has necessitated the Centers for Disease Control to initiate a health alert. The likely association of L-tryptophan ingestion with a syndrome that mimics eosinophilic fasciitis (Shulman's syndrome) further identifies an environmental agent associated with an inflammatory sclerosing rheumatic disease process. In this report, we present the clinical, morphologic, and enzyme histochemical findings in muscle, skin, and fascia biopsies from 14 cases fulfilling the Center for Disease Control diagnostic criteria for L-tryptophan-associated eosinophilia-myalgia syndrome. The clinical syndrome reveals a high incidence of arthralgia, elbow contracture, and clinical neuropathy. The absence of significant change in creatine kinase or sedimentation rate allows for diagnostic separation from other inflammatory myopathies. Histoenzymatic features in muscle biopsies reveal a preferential epimysial-perimysial noneosinophilic infiltration characterized by acid phosphatase reactive histiocytosis, nonnecrotizing venulitis, perineural inflammation within dermis and perimysium, type II fiber atrophy with superimposed denervation features, and perifascicular alkaline phosphatase reactivity representing early neofibroplasia. The constellation of changes in skin, fascia, and muscle, with the defined clinical syndrome, allows for accurate differentiation from allied syndromes, including eosinophilic polymyositis, scleroderma, idiopathic polymyositis/dermatomyositis, polyarteritis nodosa, and toxic oil syndrome. Accurate differentiation from eosinophilic fasciitis still rests on a history of L-tryptophan ingestion.
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PMID:Neuromuscular manifestations of L-tryptophan-associated eosinophilia-myalgia syndrome: a histomorphologic analysis of 14 patients. 198 74

Coxsackie B viruses are members of the family Picornaviridae which have been associated by retrospective serology with a range of muscle diseases, particularly myocarditis, dilated cardiomyopathy and epidemic pleurodynia (epidemic myalgia or Bornholm disease). It has been proposed that virus-induced myocarditis disposes to the development of idiopathic dilated cardiomyopathy. However, despite many attempts, isolation of infectious virus or immunofluorescent detection of virus-specific antigens in the affected tissue is rare, although virus may be found in faeces early in infection. This discrepancy awaited the development of nucleic acid probes to resolve the problem of whether virus was present consistently in myocardium or other muscle tissues. We report here the synthesis of Coxsackie B virus-specific complementary DNA (cDNA) probes and their use in molecular hybridizations to quantitative slot-blots of RNA prepared from either endomyocardial or skeletal muscle biopsy specimens. Of 50 patients with histologically proven myocarditis or dilated cardiomyopathy, 28 (56%) had an endomyocardial biopsy specimen positive for the presence of Coxsackie B virus-specific RNA. Twenty-two patients with other cardiac diseases of known aetiology, unrelated to virus infection, were all negative. Multiple biopsies were obtained from 20 patients with myocarditis or dilated cardiomyopathy and 15 of these (75%) had at least one biopsy specimen positive, indicating the focal nature of the disease. In analogous investigations, Coxsackie B virus-specific RNA was detected in four out of seven single skeletal muscle biopsy specimens from patients suffering from juvenile dermatomyositis, and one out of two patients with adult polymyositis. Ten muscle controls, either normal or Duchenne muscular dystrophy, were negative for virus RNA.
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PMID:The role of Coxsackie B viruses in the pathogenesis of myocarditis, dilated cardiomyopathy and inflammatory muscle disease. 284 41

We performed thallium 201 muscle scans to assess muscular involvement in 40 patients with different connective tissue diseases (7 with dermatomyositis, 7 with systemic lupus erythematosus, 12 with progressive systemic scleroderma, 2 with calcinosis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia (CREST) syndrome, 3 with monomelic scleroderma, 6 with morphea, and 3 with Raynaud's disease). Only 12 of these patients complained of fatigability and/or myalgia. Electromyography was performed and serum levels of muscle enzymes were measured in all patients. Comparison of thallium 201 exercise recording with the other tests revealed that scan sensitivity is greater than electromyographic and serum muscle enzymes levels. Thallium 201 scans showed abnormal findings in 32 patients and revealed subclinical lesions in 18 patients, while electromyography findings were abnormal in 25 of these 32 patients. Serum enzyme levels were raised in only 8 patients. Thallium 201 scanning proved to be a useful guide for modifying therapy when laboratory data were conflicting. It was useful to evaluate treatment efficacy. Because our data indicate a 100% positive predictive value, we believe that thallium 201 scanning should be advised for severe systemic connective tissue diseases with discordant test results.
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PMID:Evaluation of muscular lesions in connective tissue diseases: thallium 201 muscular scans. 337 58

Increased oxygen transport by the circulation is normally tightly coupled to increased oxygen uptake (VO2) during exercise; cardiac output (Q) increases 5 to 6 liters for every liter of increased oxygen utilization (delta Q/delta VO2 congruent to 5). We measured cardiac output and oxygen uptake at rest and during bicycle exercise in 7 patients with myalgia without evident muscle disease and 15 patients with myopathies. Resting circulation was normal in all patients, and during exercise the increase in cardiac output relative to oxygen uptake was normal in all myalgia and most myopathy patients. However, in four patients (with dermatomyositis, phosphorylase deficiency, carnitine deficiency, and ocular myopathy with "ragged-red fibers") exercise cardiac output was excessive and delta Q/delta VO2 high, resulting in an abnormally high level of cardiac work for a given level of exercise. This hyperkinetic response may represent aberrant regulation of the circulation by skeletal muscle as a consequence of some myopathies.
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PMID:Hyperkinetic circulation during exercise in neuromuscular disease. 668 23


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