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)

We report serum enzyme patterns in three patients with polymyositis or dermatomyositis whose cases posed potential problems in distinguishing malignancy, hepatocellular damage, and myocardial infarction from myositis. The alanine aminotransferase showed five- to 16-fold elevations. The creatine kinase MB isoenzyme and a predominance of lactate dehydrogenase isoenzymes 2 and 3 were present in each of the three patients. However, none of the patients showed any evidence of hepatocellular damage, myocardial infarction, or malignancy. We suggest that, although the individual occurrences are indicative of other processes, the concurrence of elevated alanine aminotransferase, creatine kinase MB, and elevated lactate dehydrogenase isoenzymes 2 and 3 is consistent with the diagnosis of polymyositis.
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PMID:Serum enzyme alterations in polymyositis. Possible pitfalls in diagnosis. 736 80

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

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

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

A 41-year-old woman developed fatal pneumomediastinum in the course of dermatomyositis. One characteristic feature of this patient was the relatively mild myositis with slight elevation of serum creatine kinase. In a literature review, some of the patients with dermatomyositis, but not polymyositis, with a very slight elevation of serum creatine kinase tended to develop spontaneous pneumomediastinum during corticosteroid therapy. Since pneumomediastinum is a highly fatal complication in patients with dermatomyositis, only slight elevation of creatine kinase could be a marker for poor prognosis. Thus, mild/minimal myositis in patients with dermatomyositis should be carefully observed irrespective of corticosteroid therapy.
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PMID:Fatal pneumomediastinum in dermatomyositis without creatine kinase elevation. 831 64

A patient fulfilling diagnostic criteria for definite polymyositis, but with normal levels of creatine kinase (CK) is reported. Review of the literature reveals significant variations regarding the frequencies of normal CK reported among cases with myositis. The discrepancies may be partly explained by inclusion of both probable and possible polymyositis, administration of steroid therapy prior to estimations of CK, and possible differences in CK levels between polymyositis and dermatomyositis. The patient reported did not suffer from neither lung disease nor concomitant malignancy. Thus, the alleged association between low levels of CK and poor prognosis in myositis could not be supported by the present case report.
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PMID:Adult idiopathic polymyositis without elevation of creatine kinase. Case report and review of the literature. 848 Jan 46


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