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)

Using an improved immunodiffusion test with partially purified antigen, PM-1 antibody was identified in the serum of 18 patients. In 67% this system was associated with a polymyositis-scleroderma overlap, it occurred less frequently in polymyositis, dermatomyositis and scleroderma, and was not detected in other rheumatic diseases. The predominant clinical features of PM-1 positive patients were muscle weakness, sclerodactyly, Raynaud's phenomenon and pulmonary disease; widespread sclerodermatous features with infrequent. Characterization of the PM-1 antigen showed it to be a heat sensitive, trypsin sensitive acidic protein associated with the cell nucleus and possibly with nucleoli.
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PMID:Clinical relevance of PM-1 antibody and physiochemical characterization of PM-1 antigen. 633 45

A 13-year-old girl with chronic graft versus host disease (GVHD) developed severe proximal weakness. Muscle biopsy showed necrotic fibers with anti-IgG deposits and interstitial inflammation. Steroid treatment helped, but her weakness recurred. Repeat biopsy showed persistent inflammation and possible steroid myotoxicity. Numerical ratio of capillaries to myofibers and capillary numerical density were higher than childhood dermatomyositis (CD), and capillaries had no tubuloreticular inclusions. Absence of quantitative histologic, ultrastructural, or immunocytochemical abnormalities of capillaries differentiated our patient from CD. We conclude that myositis of chronic GVHD can present as a severe myopathy that responds to steroids and differs from CD.
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PMID:Myositis of chronic graft versus host disease. 635 Sep 24

Twenty-seven adult patients with dermatomyositis or polymyositis were retrospectively evaluated over a mean of four and a half years. Eighty-five percent (23) met the suggested criteria of Bohan and Peter (New England Journal of Medicine, 1975) for definite or probable disease, while 15 percent had possible disease. Upper and lower limb girdle strength was given a value (0 to 5) and averaged. Initially, 26 percent were severely weak and 59 percent moderately weak. All patients were treated with steroids. Within three months, 64 percent had little to no weakness and no patients were severely impaired. These proportions remained relatively constant throughout the study. Eight less severely ill patients received alternate-day prednisone. Only two still require treatment; the remainder have been in remission for a mean period of 19 months. Of 19 who received daily prednisone, five also required cytotoxic drugs and 11 still require treatment. A comparison of the results of this study with those of major urban referral centers reveals that the patients in this study constituted a generally less ill population with a better prognosis. Forty-one percent (11) of patients were able to discontinue all forms of therapy and remain in remission. Thirty percent (eight) achieved remission with only alternate-day steroid therapy, an approach that has been discouraged in the past. Two patients (7 percent) had overt malignancies within one year before to three years after diagnosis of myositis. However, in no instance did a search for occult malignancy give positive results. Cancer detection was accomplished by following clues from history and physical examinations. It is questioned whether extensive evaluations for occult malignancies in patients with idiopathic myositis are cost-effective.
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PMID:Presentation, treatment, and prognosis of idiopathic inflammatory muscle disease in a rural hospital. 661 29

A high dose of methylprednisolone (pulse therapy) was given to 3 patients with uncontrolled dermatomyositis (DM) accompanied by severe muscle weakness. The effects of pulse therapy were compared with findings in 5 patients with severe DM who were treated with the usual dose of prednisolone (controls). The pulse therapy led to an improvement in the clinical symptoms in the 3 patients with uncontrolled DM, who were in danger of aspiration pneumonia. A rapid reduction in muscle enzyme levels in this group was also evident.
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PMID:Methylprednisolone pulse therapy in dermatomyositis. 662 98

The medical records of 47 children with dermatomyositis who were seen in the pediatric rheumatology clinic at the University of Michigan between 1964 and 1982 were reviewed. Although most children with dermatomyositis have a good prognosis, the best predictor of both good functional recovery and minimal calcinosis is early treatment after the onset of symptoms, using high doses of prednisone for an adequate length of time. Of the children given such treatment, 78% had good functional outcomes, and disabling calcinosis was seen in 20% or less. Children given treatment late in the course of disease and with low doses of steroids are more likely to be functionally limited and have a greater amount of dystrophic calcium salt deposition. In our study, only 33% of patients given such treatment had a mild disease course with good functional outcome. We have identified a subgroup of children with dermatomyositis who appear to do poorly despite optimal therapeutic regimens. These patients are distinguished by a severe disease course responding minimally to corticosteroid therapy and manifested by persistent muscle weakness, elevations of muscle enzyme activity, and severe generalized cutaneous vasculitis. These children are at high risk for the development of exoskeleton-like calcification; consideration should be given to combined immunosuppressive therapy early in the course of disease.
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PMID:Childhood dermatomyositis: factors predicting functional outcome and development of dystrophic calcification. 664 23

Weakness in polymyositis-dermatomyositis is often greater than would be suggested by histologic evidence of fiber degeneration or necrosis. Mononuclear cell infiltration is the key pathologic feature of polymyositis-dermatomyositis, and previous studies suggest a role for cellular immunity. In this study, the effect of supernatants from mitogen-stimulated human blood mononuclear cells on contractility of isolated mouse normal soleus muscle was examined. Mononuclear cell factor-rich supernatants were generated in 5-day cultures by phytohemagglutinin P stimulation of mononuclear cells from normal volunteers. Mouse soleus muscle was mounted isometrically in a muscle bath filled with oxygenated Ringer's solution or RPMI-1640 and was stimulated electrically. In 10 individual experiments, all muscles exposed to mononuclear cell factor-rich supernatants showed a rapid and progressive decline in active force of isometric contraction; a 40% decrease in active force was observed 5 to 13 minutes (average, 8 minutes) after the addition of supernatant. The inhibitory effect was reversible on removal of mononuclear cell factor-rich supernatants. Control culture medium and supernatants from unstimulated cell cultures showed no appreciable effect on active force. The factor(s) producing suppression of muscle contractility was dialyzable and stable through several cycles of freezing and thawing. Gel chromatography (Sephadex G-25 fine) showed one active fraction corresponding to an apparent molecular weight of approximately 2000. These data indicate that factor(s) released by human mononuclear cells can directly suppress the contraction of normal skeletal muscle.
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PMID:Human mononuclear cell factors suppress contractility of isolated mouse soleus muscle. 672 58

Thirteen reports of patients who developed polymyositis or dermatomyositis during treatment with D-penicillamine are reviewed and a fourteenth case is described. Twelve of the fourteen patients recovered after D-penicillamine was withdrawn; two patients died from cardiac involvement. Proximal muscle weakness was present in 13 patients and dermatomyositis in 4 patients. Dysphagia was the presenting symptom in 6 patients. Although D-penicillamine is useful in the management of rheumatoid arthritis, this drug should be used with caution and patients monitored closely for evidence of polymyositis or dermatomyositis.
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PMID:Fatal polymyositis in D-penicillamine-treated rheumatoid arthritis. 683 75

Twenty-nine patients with polymyositis and dermatomyositis seen at the three main hospitals in Auckland between 1967 and 1977 have been reviewed. Of the five diagnostic criteria used, weakness of proximal muscles (including neck flexors) was the single most important clinical diagnostic feature. The certainty of diagnosis was increased if investigations included a range of muscle enzymes, electromyography and muscle biopsy. The incidence of underlying malignancy was 10 percent. Patients with polymyositis or dermatomyositis with an associated connective tissue disorder responded better to treatment and pursued a more benign course.
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PMID:Retrospective study of polymyositis in Auckland over 10 years. 693 84

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

One hundred and seven patients with polymyositis or dermatomyositis were followed for an average of 5 years. In 100 patients it was possible to evaluate the effect of treatment with steroids or a combination of steroids and immunosuppressive drugs. Fifty patients improved, 50 did not. Compared with the treatment-resistant group the treatment-responsive patients were of younger age, did not have an associated malignancy or cardiac disease and began treatment within 24 months after the onset of muscular weakness. In the treatment-responsive group the degree of improvement was correlated to the mean dose of steroids given during the first 3 months of treatment. Eighty-seven per cent of surviving responsive patients had no or slight disability at the end of the investigation. The mortality rate was 23% and was highest in the treatment-resistant group. Comparison between different treatment programs was possible and based on the results, a treatment schedule could be recommended. The principles of this schedule are: During the first month high doses of prednisone or prednisolone should be given daily. From the second month, an alternate-day administration can be employed. Tapering to maintenance dose should be gradual and slow. The treatment period should be long. The mean duration in the present series was 27 months.
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PMID:Polymyositis--treatment and prognosis. A study of 107 patients. 710 57


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