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

We conducted a statistical review of 114 cases of dermatomyositis (DMS) treated primarily at the Department of Dermatology at Nagoya University Hospital over 27 years from 1965 to 1991 in order to determine the primary characteristics of juvenile DMS with the following results. 1) Juvenile DMS was found slightly more often in males than females; the male-to-female ratio was 1.4:1. Therefore, unlike adult DMS with its preponderance of females, there was no clear gender predominance. 2) Muscular manifestations tended to follow the appearance of cutaneous manifestations, but the frequency of minor muscular manifestations was high over the entire course of the disease. 3) Laboratory findings showed increases in serum aldolase and serum creatinine kinase with significant frequency when compared with adult patients (p < 0.01 and p < 0.05, respectively). Elevated serum aldolase most often occurred prior to or at the time of the appearance of muscular manifestations, suggesting its usefulness in early diagnosis. The positive rates for the antinuclear antibody on HEp-2 cells and anti-DNA antibody were significantly lower in children than in adults (p < 0.001 and p < 0.05, respectively). 4) There were no cases of juvenile DMS complicated by malignant tumors, interstitial pneumonia, or pulmonary fibrosis. There were also no deaths, and the rate of "remission or improvement" was significantly higher than in adult DMS cases (p < 0.05). Adult cases which remained the same or worsened usually presented with intractable muscular manifestations. In children, however, the cutaneous manifestations were more difficult to treat.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Juvenile dermatomyositis: a statistical study of 114 patients with dermatomyositis. 140 7

Twenty-nine patients with myositis, including 10 with polymyositis (PM), 6 with dermatomyositis (DM), and 13 with myositis associated with a connective tissue disease (CTD), were followed up for a mean observation time of 49 months. The 13 patients with CTD-associated myositis were further separated by the presence or absence of anti-RNP antibodies. The functional disability at diagnosis was pronounced without differences between the groups. The patients with anti-RNP antibodies did not differ from the other patients regarding initial muscle weakness, erythrocyte sedimentation rate, or creatinine phosphokinase values, but the histopathological muscle changes were generally milder. Rapid improvement of muscle strength on moderate doses of corticosteroids was seen in most patients. At the end of study, corticosteroid treatment had been withdrawn from 17 patients because of remission, including 6 of the 7 patients with anti-RNP antibodies. The presence of electromyographic changes compatible with myositis, pronounced muscle weakness before treatment, and a low erythrocyte sedimentation rate seemed to indicate a less favorable outcome unrelated to diagnostic subgroupings. However, the combination was rarely found among the anti-RNP-positive patients.
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PMID:A clinical, serological, and histopathological study of myositis patients with and without anti-RNP antibodies. 143 44

Renal involvement is usually considered to be rare in dermatomyositis and polymyositis. The aim of this prospective study was to reevaluate the renal changes which may occur in these two conditions. Twenty patients, 12 with dermatomyositis and 8 with polymyositis of the adult type, were investigated to determine the incidence, the severity and course of renal disease. The 24 hour proteinuria was over 0.1 g in 14 cases and greater than 1 g/24 hours in 6 cases, including one patient with the nephrotic syndrome. Microscopic haematuria was noted in 3 cases and pyuria in 8 cases. Endogenous creatinine clearance was significantly decreased (p less than 0.001) in patients with dermatomyositis compared with a control group of subjects paired for age, sex, weight and serum creatinine concentration. Renal biopsy (5 cases) showed minimal glomerular lesions (2 cases), endocapillary proliferation and extramembranous glomerulonephritis (1 cases) and amyloid deposits (1 case). Although the proteinuria, haematuria and pyuria responded to steroid therapy (except in 1 case), the creatinine clearance remained decreased during the course of steroids.
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PMID:[Renal manifestations in dermatomyositis and polymyositis]. 357 91

Cyclosporine is an immunosuppressive agent which is well-established in the transplantation of organs including kidney, liver and bone marrow. It acts by inhibiting the production of interleukin 2, thereby blocking both the development of cytotoxic lymphocytes, and the proliferation of helper T cells. T cell-mediated muscle damage is thought to be important in the pathogenesis of polymyositis. And activated cytotoxic T cells are thought to play an important role of polymyositis/dermatomyositis with active pneumonitis. It is thereby likely that cyclosporine would be effective in the management of polymyositis with interstitial pneumonitis. We have used cyclosporine in two cases of corticosteroids resistant polymyositis associated with pneumonitis. The first case was admitted because of the relapse of polymyositis. She was partially responded by the high dose of steroid, but showed decreased %DLCO and increased AaDO2 during the therapy. And oral cyclosporine was given with steroid. Within two weeks, serum creatinine kinase level was reduced to normal range, and the improvement of pneumonitis was observed. The second case was admitted because of the flare of pneumonitis. She was treated with high dose of steroid with insufficient response. And cyclosporine was prescribed. Within two weeks of treatment, her symptom was relieved, and blood gas analysis showed an improvement of pulmonary function. And steroid could be tapered. In both cases, the initial dose of cyclosporine was 200 mg/day, and the optimal trough level was thought to be ranged 100 to 150 ng/ml. In the second case, renal dysfunction was observed but it was recovered by the reduction of the dose of cyclosporine. No other side effect was appeared.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Successful combination therapy of cyclosporine and steroids in two cases with interstitial pneumonitis associated with polymyositis]. 773 99

Demonstration of myositis in juvenile dermatomyositis (JDM) has traditionally required the presence of two out of three objective findings including muscle enzyme studies, electromyography, and muscle biopsy. Magnetic resonance imaging (MRI) is considered another objective criterion and has been recommended for follow-up studies as well. We report on a 6-year-old girl with JDM, presenting with facial rash and proximal muscle weakness, in whom MRI, in conjunction with elevated creatinine kinase (CK) levels, was diagnostic, with an increased T2 signal of proximal muscles. MRI reflected exacerbation of myositis in spite of steroid therapy 2 weeks later, but failed to mirror normalization of muscle strength and CK values 10 weeks later. In fact, improvement of MRI followed clinical and laboratory normalization with a delay of 2 months. This possible delay should be considered when MRI is used to monitor the response to therapy.
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PMID:Serial magnetic resonance imaging in juvenile dermatomyositis--delayed normalization. 783 73

In the last few years high-dose intravenous therapy with gammaglobulin in juvenile dermatomyositis with steroid resistant myopathy has been discussed as an alternative to cytostatic therapy. The 12-year-old Filipinogirl suffered from dermatomyositis with pronounced weakness of the limbs and masticatory muscles; creatinine-kinase was increased 22-fold. After 5 weeks of therapy with prednisolone (1.5 mg/kg body/weight/daily), the creatinine kinase decreased to sixfold of its standard value; the muscular weakness, however, progressed to almost complete paralysis of the scapular abductors and rotators and the coxofemoral flectors and rotators. Additional therapy with gammaglobulin at a dose of 1 g/kg body weight on two consecutive days at 4-week intervals led to complete restoration of muscular strength, normalization of muscle enzymes, and stabilization of these results after 12 courses of gammaglobulin and discontinuation of the steroid medication after 8 courses. This observation suggests that high-dose intravenous application of gammaglobulin represents an efficient therapy with minimal side effects in steroid-resistant juvenile dermatomyositis.
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PMID:[High dosage intravenous gamma globulin therapy in juvenile dermatomyositis]. 784 65

Two women, aged 72 (case 1) and 77 (case 2) were referred for neurological diagnosis because of progressive muscular weakness, for 4 and 18 months, respectively, which had finally led to dysphagia and required mechanical ventilation. The cause of the disease in case 1 was classical dermatomyositis. Creatinine kinase concentration, never previously measured, was 950 U/l. In case 2 there was polymyositis, previously not considered as she had a diabetic polyneuropathy and muscle enzyme concentration was normal. In both cases the correct diagnosis was speedily confirmed by electromyography and muscle biopsy. Immunosuppressive treatment was largely successful in reversing the symptoms (case 1: initially 500 mg methylprednisolone daily, reduced to 50 mg daily within 6 weeks; case 2: 500 mg methylprednisolone daily for 1 week, then 100 mg daily plus 150 mg azathioprine with maintenance dosage of 20 mg glucocorticoid daily).--These two cases demonstrate that, particularly in the elderly, dermatomyositis and polymyositis should be considered in the differential diagnosis of progressive general weakness.
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PMID:[Is chronic dermato- or polymyositis misdiagnosed as "weakness of old age"?]. 822 97

A 61 year old man complained initially about pain in the left lower leg with increasing swelling of the entire left leg. A slight swelling of the left ankle and knee and an erythematous skin change of 5 cm diameter developed and disappeared after a few hours. Two days later the patient complained about a sudden and massive weakness of the proximal skeletal muscles, progressive disturbance of swallowing, hoarseness and dark red to bluish erythematous skin eruptions on the right knee and buttock. An elevated creatinine-kinase and lactate-dehydrogenase were found. Dermatomyositis was diagnosed and treatment with high dose steroids initiated. Under this treatment the patients condition deteriorated dramatically. A treatment with plasmapheresis and cyclophosphamide was begun. A significant improvement occurred over three weeks. The patient was discharged, rehabilitated and remains free of symptoms on 10 mg cortisone. A neoplasm has so far not been detected.
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PMID:[Muscle weakness]. 826 41

Although cardiac abnormalities have been reported in patients with idiopathic polymyositis-dermatomyositis (PM), the nature and extent of these abnormalities have varied. The purpose of this study was to determine the prevalence and to obtain a better description of the spectrum of cardiac abnormalities in a cohort of patients with PM by use of a thorough noninvasive cardiac evaluation. Accordingly, we studied 26 patients with a history of PM and clinically inactive myositis (22 polymyositis, 4 dermatomyositis) between June 1984 and June 1991. Examination included history, physical examination, 12-lead electrocardiography, 24-h ambulatory electrocardiographic monitoring, chest radiography, transthoracic echocardiography, and radionuclide ventriculography. Of the patients studied, 77% were taking corticosteroid medications at a mean dose of 39 +/- 27 mg at the time of their evaluation. All 26 patients were identified as having two or more cardiac abnormalities. Cardiac symptoms and signs were common (62 and 81%, respectively), but were generally nonspecific. Electrocardiographic findings were most common (in 85% of cases), followed by findings on ambulatory monitoring (77%), echocardiography (42%), and radionuclide ventriculography (15%). The prevalence of mitral valve prolapse (8%) and hyperkinetic left ventricular contraction (12%) was significantly lower than previously reported. A secondary aim of this study was to determine associations between demographic variables including age, disease duration, cardiovascular symptoms, immunosuppressive therapy, autoantibody status, and creatinine phosphokinase level, and the presence of cardiac abnormalities. Of these patient variables, only increasing patient age was associated with an increased likelihood of cardiac abnormalities on noninvasive testing.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The heart in polymyositis: a prospective evaluation of 26 patients. 826 58

We report the case of a 42-year-old woman with concomitant panniculitis and dermatomyositis. Painful, indurated lesions on the buttocks, thighs, arms, abdomen and breasts were associated with proximal muscle weakness. Skin biopsy revealed lobular panniculitis, and vacuolar degeneration of epidermal basal cells. Direct immunofluorescence was negative. Serum muscle enzyme (creatinine-phosphokinase) levels were elevated, and electromyography demonstrated a myositic process. Muscle biopsy showed an inflammatory myositis. These results were consistent with dermatomyositis associated with panniculitis. Only five cases of this association have been reported previously. The relationship between these two conditions is discussed.
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PMID:Subcutaneous changes in dermatomyositis. 849 60


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