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

Few guidelines exist for the use of corticosteroid therapy in polymyositis-dermatomyositis (PM-DM). We retrospectively examined the relationship between serum creatine kinase (CK), muscle strength and the dosage and method of administration of prednisone in 30 patients with PM-DM observed monthly for a minimum of one year. Forty-two corticosteroid treated episodes of proximal muscle weakness associated with CK elevations formed the final study group. Each patient course was designated as having a good or poor biochemical and clinical outcome based on predetermined criteria. Adherence to 3 principles predicted a favorable biochemical and clinical outcome in the treatment of myositis: (1) administration of an adequate initial (loading) corticosteroid dose; (2) continuation of the initial dose until or after the time that the serum CK had become normal; and (3) a slow corticosteroid taper rate. Achievement of a CK within the low normal range predicted a prolonged biochemical remission, and a rise of CK within the normal range signalled a subsequent biochemical and clinical relapse. Tapering the corticosteroid dose when the CK was elevated frequently resulted in a further increase in CK. These observations allowed us to develop practical guidelines for the management of PM-DM.
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PMID:Relationship between serum creatine kinase level and corticosteroid therapy in polymyositis-dermatomyositis. 317 94

A patient presenting with interstitial lung disease was predicted to have underlying polymyositis when found to be anti-Jo-1 antibody positive. When myopathy developed later with elevated serum creatine phosphokinase and myoglobin levels, the electromyogram was nonmyopathic, and polymyositis was confirmed by muscle biopsy. The case illustrates the value of anti-Jo-1 as a 'marker' autoantibody for polymyositis, and the need for thorough investigation of patients with interstitial lung disease of uncertain etiology. This should include indirect immunofluorescence using a dividing cell substrate and tests for antibodies to extractable tissue antigens. The need to completely evaluate patients with dermatopolymyositis is also emphasised.
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PMID:Interstitial lung disease as the presentation of anti-Jo-1 positive polymyositis. 349 60

Sixteen cases of dermatomyositis-polymyositis in children (dermatomyositis 15; polymyositis 1) have been reviewed. Proximal muscle weakness with a characteristic skin rash was vital for diagnosis in the majority of children. The positive laboratory tests such as creatine phosphokinase (CPK), electromyography (EMG) and muscle biopsy were helpful but results were normal in some cases even in the acute phase of the illness. All but one had been treated initially with high dose corticosteroids followed by a low dose maintenance for a prolonged period. Two in addition had had cytotoxic agents. Thirteen of the sixteen were currently in remission. In the other three cases, one with polymyositis only, the disease remained active and the two with dermatomyositis died. Only three of the patients were incapacitated by residual fixed contractures and extruding calcinosis. On the whole the prognosis of dermatomyositis is good.
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PMID:Dermatomyositis-polymyositis in children. 351 51

Twenty-five patients with adult idiopathic dermatomyositis/polymyositis were followed for 59 +/- 39.3 months from initiation of therapy. Forty-two percent of the patients were able to discontinue prednisone therapy after 56 +/- 33 months of therapy and remained disease free for 34 +/- 35.2 months after discontinuing therapy. The mean dose of prednisone in 14 patients being treated at the end of the follow-up period was 16.0 mg. A positive correlation between age at diagnosis and duration of therapy was noted, but not with the creatine kinase value at diagnosis. Sex and dermal involvement were also not associated with treatment duration. All seven patients who received immunosuppressants continued to receive treatment during the follow-up period.
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PMID:Influence of age at onset on the duration of treatment in idiopathic adult polymyositis and dermatomyositis. 367 1

Strength of the quadriceps and hamstring groups was biomechanically assessed in terms of isometric torque production in 14 patients with inflammatory myopathy. Eleven had polymyositis and 3, dermatomyositis. Determinations of serum creatine kinase, lactate dehydrogenase, transaminase, and myoglobin were simultaneously obtained over an average period of observation of 1.8 years. In certain individual patients, there were significant correlations between laboratory indices and strength during the entire course of illness. In others, this was not the case. In the total group of patients, absolute values of the laboratory indices did not correlate well with strength except in the case of serum myoglobin, where there a significant inverse relationship. Logarithmic transformations of the laboratory data increased the inverse correlations. High strength and low myoglobin were related to high prednisone dose. Since laboratory guides are not always related to disease activity, quantitative assessment of muscle strength is necessary.
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PMID:Serum enzyme, myoglobin and muscle strength relationships in polymyositis and dermatomyositis. 372 97

The clinical and serologic features of 36 patients with polymyositis (PM) or dermatomyositis (DM) were observed over a 5-year period. The mean age of the patients at the time of diagnosis was 48.5 years, and 61% were female. According to widely accepted diagnostic criteria 50% had PM (group I), 14% DM (group II), 11% PM or DM associated with malignant disease (group III) and 25% PM or DM associated with a connective tissue disorder (group V). None of the patients had childhood PM or DM associated with vasculitis (group IV). All the patients had muscle weakness, and 94% of the patients tested had an elevated serum level of creatine kinase. The average delay from the onset of symptoms to diagnosis was 14 months overall but only 2.3 months for the DM patients. Of the 30 patients whose serum was tested, 73% had antinuclear antibodies, with antibodies to nuclear ribonucleoprotein being most common in group V patients and antibodies directed against the Jo-1 antigen being restricted to patients with PM alone (group I).
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PMID:Clinical and serologic features of patients with polymyositis or dermatomyositis. 387 56

Serious cardiac abnormalities and asymptomatic hyperkinetic heart function are frequently seen in patients with polymyositis/dermatomyositis (PM). The cardiac manifestations may be secondary to myocardial inflammation, replacement fibrosis, or alterations in the peripheral circulation. The significance of determinations of cardiospecific isoenzymes in the evaluation of myocardial manifestations in PM has not yet been established. We report on 2 patients with firm evidence of severe myocardial involvement. Nevertheless, the serum levels of creatine phosphokinase MB (CPK MB) and lactated dehydrogenase isoenzyme 1 (LDH1) were found normal. This finding casts doubt on the reliability of cardiospecific isoenzymes for the identification of inflammatory myocardial involvement in patients with PM.
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PMID:Creatine phosphokinase MB and lactate dehydrogenase isoenzyme 1 in polymyositis. 408 64

An increased frequency of diffuse interstitial lung disease (DILD) was found in 7 of 15 cases of polymyositis and dermatomyositis seen in a 7-yr period. Affected patients had higher values of creatine phosphokinase and aldolase and more severe muscle necrosis than 8 patients with DILD. Early corticosteroid therapy sufficient to normalize enzymes apparently led to better resolutions of DILD. Active muscle necrosis seemed to be related to the development of DILD.
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PMID:Interstitial lung disease in polymyositis and dermatomyositis. 696 22

Eighteen adults were studied an average of 18.5 years after the diagnosis of juvenile dermatomyositis had been made. Persistent weakness was found in six patients and recurrent rash in the classic distribution in seven. Other clinical features present were Raynaud's phenomenon, arthritis and subcutaneous nodules. Minor increases in the serum creatine phosphokinase level were noted in seven patients and did not correlate with the presence of weakness or rash. Educational achievement and employment status were better in this group than in a group of adults with juvenile rheumatoid arthritis or the general adult population of British Columbia. Significant residual disability related to calcinosis and flexion contractures was present in only three of the patients.
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PMID:Juvenile dermatomyositis: medical, social and economic status in adulthood. 705 70

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


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