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

Juvenile dermatomyositis (JDM) is characterized by microvasculopathy of the striated muscle, which indicates different etiology, clinical manifestation and prognosis from the adult-onset dermatomyositis. We experienced 10 cases of JDM and 1 case of juvenile polymyositis (JPM) in the recent 14 years, and analyzed clinical manifestation, laboratory findings, treatment anrognosis. The cases were 9 girls and 2 boys. The onset of the disease was 2 years of age in 2 patients, and 9 to 13 years of age in 9 patients. During the follow-up courses, no cases were dead or complicated with neoplasm. Skin rash was the most frequent manifestation at the onset, and facial erythema was common. Muscle weakness was observed only in 4 cases at the onset, and in all cases muscle enzymes including creatine kinase and aldolase were elevated. The clinical course was classified into three groups; monocyclic (5 cases), chronic and recurrent (4 cases), and fulminant (2 cases). Prognosis depended not on the degree of the elevated serum muscle enzymes, but on the initial therapy employed at the onset of the disease. Five cases including 2 cases of fulminant type were initially treated with methylprednisolon pulse therapy, and all of these had no recurrence. On the other hand, 6 cases were started the therapy with p.o. prednisolone. Four of them had frequent recurrences in accordance with tapering of prednisolone. These cases were effectively treated with the combination with immunosuppressants. In previous reports, JDM and JPM were reported to be a disorder which had relatively favorable prognosis. But we found that one third of the cases had chronic and recurrent courses. Methylprednisolone pulses as initial therapy may be effective in preventing the chronicity and recurrence of the disease.
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PMID:[Clinical analysis of 11 cases of juvenile dermatomyositis and polymyositis]. 1004 16

The clinico-laboratory features of 16 patients with dermatomyositis (DM) were compared between patients with accompanying rapidly progressive interstitial lung disease (RP-ILD, n = 7) and those with chronic interstitial lung disease (C-ILD, n = 9), and also between deceased (seven RP-ILD and three C-ILD) and living patients (six C-ILD). The extent of muscle weakness of the extremities and frequency of autoantibody positivity were significantly lower in DM patients with RP-ILD than in DM patients with C-ILD. Furthermore, significantly lower serum creatine kinase/lactate dehydrogenase levels (0.26+/-0.27) were found in the 10 patients who died than in the six living patients (1.21+/-1.09). A higher CD4+/CD8+ T-lymphocyte ratio in the peripheral blood (3.51+/-2.65) was detected in the four DM patients with RP-ILD who died than in the six living DM patients with C-ILD (1.22+/-0.49).
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PMID:Clinico-laboratory characteristics of patients with dermatomyositis accompanied by rapidly progressive interstitial lung disease. 1063 71

An open study was conducted to identify and investigate dermatomyositis patients who benefit from IVIG treatment, based on dermatological criteria, myositis-related symptoms and immune/inflammatory parameters. 19 patients (16 females and three males, ages 31-84) suffered from dermatomyositis, and 4/19 patients had paraneoplastic dermatomyositis. We monitored the disease activity by documenting the clinical symptoms, recording muscle-related parameters (electromyography, serum creatine kinase, histopathology), and by determining circulating autoantibodies and serum levels of IL-6, sIL-2R, sTNF-a-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate muscle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders had severe skin and muscle disease, concomitant with autoantibodies and/or malignancy. sIL-2R levels were initially elevated in all patients but reverted to normal in IVIG-responders only. Creatine kinase-levels and other parameters did not correlate with disease activity and/or treatment response. IVIG is effective in selected dermatomyositis patients. sIL-2R serum levels appear to be useful predictors of IVIG-induced treatment response and disease activity.
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PMID:High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels. 1069 94

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
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PMID:Dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. 1071 98

Thirty-six patients with juvenile dermatomyositis, seen consecutively between 1983 and 1996 and treated initially with low doses of corticosteroids (prednisolone 1 mg/kg/day), were studied retrospectively to evaluate their long-term evolution and to identify factors predictive of the functional outcome. After a mean follow-up of 4.9 years, 28 (78%) of the patients were well without functional impairment; five patients had inactive disease but with persisting disabilities; and three patients had active disease despite several years of treatment. Fifteen children (42%) developed dystrophic calcifications which, in five of these patients, interfered with functions. These patients treated for juvenile dermatomyositis with a low dose corticosteroid regimen had an evolution identical to that of the published series of patients treated with higher doses and probably had a better quality of life. The best predictors of good functional recovery and minimal calcinosis were early treatment after the onset of symptoms and low creatine kinase serum level at the time of diagnosis.
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PMID:Childhood dermatomyositis: clinical course of 36 patients treated with low doses of corticosteroids. 1072 93

Five cases of dermatomyositis in four Shetland sheepdog puppies and one adult bitch are described. The dogs all had well-defined patches of scaling, crusting and alopecia over the muzzle, periorbital skin and distal limbs, and the tail, perineum and pinnae were affected in some of them. The affected puppies were all sired by the same stud dog. The affected adult bitch was unrelated to the puppies. Three of the four dogs tested had high serum creatine kinase concentrations and electromyographic abnormalities were detected in three of the four dogs tested. The histological changes observed in the skin of four of the dogs strongly supported the diagnosis of dermatomyositis, and in the fifth dog they were compatible with this diagnosis. Two of the puppies were euthanised shortly after being diagnosed. In the other two puppies and the adult the disease remains stable and non-progressive 15 to 18 months after diagnosis. The sire of the four affected puppies has been used extensively because it was considered to be genetically clear of collie eye anomaly.
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PMID:Dermatomyositis in five Shetland sheepdogs in the United Kingdom. 1073 Oct 70

Dermatomyositis (DM) is a rare inflammatory disorder of the skin and muscles associated with an increased incidence of malignancy. We describe herein the case of a 59-year-old woman with DM accompanied by rectal cancer. Following excision of the rectal cancer, the characteristic features of the skin rash such as the heliotrope eyelid rash and Gottron's papules, and proximal muscle weakness, improved. Moreover, the elevated preoperative serum levels of muscle-associated enzymes, including aspartate transaminase, creatine phosphokinase, lactate dehydrogenase, and aldolase, decreased from 38 to 16 (IU/1), 138 to 42 (IU/1), 672 to 515 (IU/1), and 32.2 to 4.3 (IU/1), respectively. The current concepts of the correlation between DM and malignancy are discussed with regard to the present case.
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PMID:Dermatomyositis accompanied by rectal cancer: report of a case. 1075 89

The objective was to investigate whether a 12-week resistive home exercise program in addition to conventional medical treatment could be safely performed regarding muscle inflammation, muscle function, and quality of life in patients with active polymyositis (PM) or dermatomyositis (DM). Eleven patients diagnosed with active PM or DM were included. Muscle biopsies and Magnetic Resonance Imaging (MRI) of the thighs were performed. Quality of life, function, and subjective global disease impact (SGDI) were assessed and creatine phosphokinase levels (CPK) were analysed. The patients exercised with the exercise program for 15 minutes and took a 15-minute walk five days a week for 12 weeks. After the exercise period there was no sign of increased muscle inflammation. The group showed significantly improved function and quality of life compared to the start of study. It seems that this exercise program safely can be employed in patients with active PM or DM, and we suggest that physical exercise should be included in the rehabilitation of these patients.
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PMID:The safety of a resistive home exercise program in patients with recent onset active polymyositis or dermatomyositis. 1109 95

A 20-year-old woman initially presented with edematous erythema in the upper eyelids in December 1995. She was admitted to our department in January 1996 because of fever and multiple arthralgia. She was given a diagnosis of dermatomyositis (DM) on the basis of characteristic eruption, elevated serum level of creatine kinase, and increased inflammatory reaction. Chest computed tomography (CT) revealed faint interstitial changes in the left lower lung. The administration of corticosteroid caused improvement in the patient's condition and the interstitial lesion in the lung. Although she was asymptomatic, chest CT showed pneumomediastinum in the pretracheal space and concomitant pneumothorax around left bronchus. Those changes spontaneously disappeared 4 weeks later without treatment. The occurrence of pneumomediastinum in patients with DM has been well documented as an indicator of poor prognosis in the literature. It seems that a severe pulmonary disorder could secondary cause pneumomediastinum. In our case, however, the pneumomediastinum developed in spite of the low grade severity of the pulmonary lesion. This finding suggested that the pneumomediastinum may be associated with the development of DM itself.
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PMID:[A case of dermatomyositis associated with asymptomatic pneumomediastinum]. 1115 94

The purpose of this study was to evaluate the clinical significance of magnetic resonance imaging (MRI) of skeletal muscles in Japanese patients with idiopathic inflammatory myopathies (IIM). MRI was performed in 23 adult patients with IIM, including 10 with polymyositis, 12 with dermatomyositis, and 1 with focal myositis. Seven (73%) of 11 patients with active IIM and 2 (17%) of 12 patients with inactive IIM showed hyperintensity of T 2-weighted images and normal intensity of T 1-weighted images, indicating "edema-like abnormalities" (MRI findings for active myositis). Muscle lipomatosis and fibrosis were demonstrated in four patients and 1 patient, respectively. Considerable selectivity of muscles in developing inflammatory disorders was found. In quadriceps muscles, for example, vastus muscles seemed to be more often affected in DM patients, whereas adductors were more often affected in PM patients. Serial examination of muscle MRIs was carried out in 4 patients and the findings paralleled the disease activities. The muscle MRI findings did not necessarily correlate with other findings, such as the presence of muscle weakness, elevated serum creatine kinase levels, myogenic electromyogram, or muscle biopsy findings. The muscle MRI was considered to be an additional useful tool for the diagnosis, evaluation of disease activity, and planning treatment of IIM.
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PMID:[Clinical significance of magnetic resonance imaging of skeletal muscles in idiopathic inflammatory myopathies of adults]. 1121 Jul 72


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