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)

Twenty five patients with idiopathic myositis attended this department for long term follow up from 1980 to 1989. Twelve patients had primary polymyositis (four men, eight women) and six had primary dermatomyositis (three men, three women); five women had an overlap syndrome. Two patients had a malignant condition associated with the myositis. The mean age at diagnosis was 40 years. All of the patients had proximal muscle weakness, 18/25 had a raised creatine kinase value (mean 2325 IU/l), 19/20 had an abnormal electromyogram, and 19/24 had positive muscle biopsy samples. Of the disease specific antibodies, anti-Jo-1 was detected in only 1/21 patients tested (three patients with fibrosing alveolitis were negative for this antibody), but the 56 kDa antibody was detected in 12/17 patients. The HLA data analysed in the white patients (17/25) showed that 6/8 of those tested were HLA-DR3 positive. All patients were treated with prednisolone and azathioprine was used for 14/25 patients. Only three deaths occurred during the eight year follow up, but there was a substantial morbidity, which may reflect the referral pattern. Muscle strength tests and creatine kinase levels were useful in recording the response to treatment in some patients. These data emphasise that careful long term follow up of patients with myositis is mandatory and that although the present treatment strategy has substantially reduced the death rate, morbidity associated with the disease remains a major problem.
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PMID:Idiopathic myositis: a rheumatological view. 154 36

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

A correlation study was performed on the degree of muscle weakness in 36 patients with dermatomyositis and 69 with polymyositis in relation to muscle biopsy findings, electromyography (EMG) abnormalities, and serum concentrations of creatine kinase (CK), aspartate aminotransferase (AST) and alanine aminotransferase (ALT) enzymes. Statistically significant correlations were found between muscle weakness and EMG results in patients with polymyositis, and between muscle weakness and serum CK and AST levels in dermatomyositis. As expected, correlations were found between the results of the three enzyme determinations in both groups of patients.
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PMID:Correlation between tests of muscle involvement and clinical muscle weakness in polymyositis and dermatomyositis. 208 50

Cases of polymyositis or dermatomyositis in which levels of all the serum muscle enzymes are within the normal range throughout the entire clinical course are very rare. It has recently been suggested that when there is no increase in creatine kinase the prognosis in dermatomyositis is poor and there is a higher incidence of malignancy and interstitial lung disease associated with the condition. We describe a rare case of dermatomyositis without increase in serum muscle enzymes, including creatine kinase. No evidence of malignancy or interstitial lung disease was found after a followup of 18 months. In accordance with the report of a similar case, we suggest that the absence of an increase in creatine kinase in dermatomyositis is not necessarily a poor prognostic sign.
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PMID:[Dermatomyositis associated with normal serum muscle enzyme levels]. 274 49

During the last several years, there has been increasing interest in the use of intravenous immune globulin for immunosuppression. Although the mechanism(s) of action remains to be delineated, immune globulin therapy has been shown to be effective in some antibody-mediated disorders. In Rh disease, antibody-induced cytopenias, myasthenia gravis, and the clotting disorder associated with anti-factor VIII antibody, intravenous immune globulin has had therapeutic benefit. It was of interest that intravenous immune globulin may partially ameliorate the tendency toward dilation of the coronary vessels in Kawasaki disease. If this disorder represents a vasculitis of the small feeding vessels of the coronary arteries, could this agent influence other forms of collagen vascular disease? Pilot studies in dermatomyositis and polymyositis and systemic juvenile rheumatoid arthritis indicate benefit from intravenous immune globulin therapy. In these studies we used a high-dose protocol consisting of 1 gm/kg/day of immune globulin for 2 days every 4 weeks. In patients with either myositis or systemic juvenile rheumatoid arthritis, beneficial effects were seen. In the former group of patients, increased proximal muscle strength and reduction in creatine kinase levels were observed. In the latter group of patients a marked reduction in systemic features was observed. The amount of corticosteroids required was reduced in both groups of patients. These studies indicate the potential for intravenous immune globulin in collagen vascular disorders and the need for carefully controlled trials of this form of therapy.
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PMID:The use of intravenous immune globulin in collagen vascular disorders: a potentially new modality of therapy. 279 2

Serum muscle enzyme levels are usually elevated in patients with untreated polymyositis and dermatomyositis. Creatine kinase is the muscle enzyme most often used to diagnose inflammatory myopathies. Seven patients with dermatomyositis and normal creatine kinase levels are described. Five of the seven patients had either an associated malignancy or severe interstitial lung disease. The one-year survival of the six patients followed for that length of time was 33 percent. A lack of creatine kinase elevation in patients with dermatomyositis is a poor prognostic sign.
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PMID:Dermatomyositis without creatine kinase elevation. A poor prognostic sign. 300 9

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

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


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