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

Total creatine activity was greatly increased, and creatine kinase MB isoenzyme was measurable in the serum of a patient with dermatomyositis. In contrast, total CK activity in an extract of skeletal muscle from this patient was markedly decreased as compared with normal muscle extracts, but contained about the normal proportion of the MB isoenzymes, 1.5%. We believe skeletal muscle to be the source of the MB isoenzyme in the serum, because the patient's electrocardiographic pattern was normal and she had no signs or symptoms of myocardial infarction.
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PMID:Total creatinine kinase and isoenzyme MB activity in serum and skeletal muscle of a patient with dermatomyositis. 743 45

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

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

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years (range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.
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PMID:Dermatomyositis and polymyositis in childhood. 794 27

The purpose of the study was to describe typical MRI findings in various types of idiopathic inflammatory myopathies in adulthood and to correlate the MRI with histopathological and electromyographic findings, and the serum creatine kinase (CK) activity. A third goal was to assess the diagnostic value of the use of gadolinium-DTPA (Gd-DTPA). Fifty-eight patients (35 women, 23 men), aged 21-83 years (median age 59 years), suffering from idiopathic myositides (13 with acute and 45 chronic diseases; 25 with polymyositis, 14 with dermatomyositis, 8 with granulomatous and 11 with inclusion body myositides) were examined with MRI. Seventeen of them received an intravenous infusion of Gd-DTPA. Histopathological and MRI findings of 21 muscles of 18 patients were compared. MRI of skeletal muscles showed abnormal signal intensities in 56 (96.6%) of the 58 patients. MRI abnormalities were found more often than elevated CK activity (P < 0.001). The hyperintensity of T2-weighted images was more conspicuous than on T1-weighted images in 26 (44.8%) patients, indicating oedema-like abnormalities. MRI of 50 (86.2%) patients showed fat replacement. In acute myositides, oedema-like abnormalities were more often visible and in muscle lipomatosis less often visible than in chronic diseases (P < 0.05 each). In dermatomyositis oedema-like abnormalities were more and lipomatosis less frequent than in the other types of myositis (P < 0.005) and correlated with the acuteness of the disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Magnetic resonance imaging of skeletal muscles in idiopathic inflammatory myopathies of adults. 800 84

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

A 29-year-old woman with quiescent Crohn's disease developed severe myositis. Serum creatine phosphokinase was markedly elevated, and muscle biopsy showed typical findings of dermatomyositis. The muscular symptoms promptly responded to combined treatment with corticosteroids and azathioprine. The association between inflammatory bowel disease and inflammatory myopathies is very rare. We review the clinical, pathological, and pathophysiological aspects of this association.
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PMID:Dermatomyositis associated with Crohn's disease. 811 86

Myositis due to the etiologic agent of Lyme disease, Borrelia burgdorferi, has been reported nine times in the English-language literature; there has been but a single report of exacerbation of dermatomyositis due to B. burgdorferi in a patient with known dermatomyositis. Multiple infectious agents, but not B. burgdorferi, have been hypothesized to trigger dermatomyositis. We report the first case of dermatomyositis that appears to have been triggered by B. burgdorferi. This case involved an individual from Westchester County, NY, who presented with skin lesions suggestive of erythema migrans and who was seropositive for Lyme disease. He soon developed a clinical syndrome suggestive of dermatomyositis: periorbital edema, dysphagia, proximal muscle weakness, and a markedly elevated level of creatine phosphokinase. We also review the clinical presentation and response to treatment of patients with Lyme myositis.
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PMID:Dermatomyositis associated with Lyme disease: case report and review of Lyme myositis. 816 22

Dermatomyositis (DM) developed in a 35-year-old woman after 2 months of psoralen ultraviolet-A (PUVA) therapy for presumed psoriasis. Her disease was characterized by symmetrical proximal muscle weakness and cutaneous lesions compatible with DM. In addition, laboratory abnormalities included a positive antinuclear antibody, and elevated levels of creatine kinase and aldolase. This is the first report of DM developing during PUVA therapy.
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PMID:Dermatomyositis occurring during psoralen A (PUVA) therapy. 818 50

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


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