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

To evaluate the value of myosonography in inflammatory myopathies ultrasound of skeletal muscles was performed in 70 patients, aged 21-82 years, suffering from histologically proven polymyositis (n = 30), dermatomyositis (n = 18), granulomatous myositis (n = 9), inclusion body myositis (n = 13), and in 102 control persons. The sensitivity of muscle ultrasound in detecting histopathologically proven disease (82.9%) was not significantly different from electromyography (92.4%) or serum creatine kinase activity (68.7%). The positive predictive value of ultrasound was 95.1%, the negative predictive value 89.2%, and the accuracy 91.3%. The different types of inflammatory myopathies presented with typical, but not specific ultrasound features. Polymyositis showed atrophy and increased echointensity predominantly of lower extremity muscles, whereas in dermatomyositis clear muscle atrophy was rare and echointensities were highest in forearm muscles. Echointensities were lower in dermatomyositis compared to poly- and granulomatous myositis. Granulomatous myositis was characterized by the highest echointensities and a tendency towards muscle hypertrophy. Severe muscle atrophy was the most impressive feature in the majority of patients with inclusion body myositis. Comparison of ultrasound and histopathological findings indicates that muscle lipomatosis has a much greater impact on muscular echointensity than does muscle fibrosis. Ultrasound of myositis improved clinical assessment of patients by supplying differential diagnostic clues based on precise muscle size measurements and identification of mesenchymal abnormalities, particularly muscle lipomatosis.
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PMID:Muscular ultrasound in idiopathic inflammatory myopathies of adults. 850 7

An autopsy case of dermatomyositis with rapidly progressive interstitial pneumonia is reported. a 48-year-old woman was admitted because of facial edematous erythema and muscular weakness. A diagnosis of dermatomyositis was made because of typical erythema and myogenic pattern of EMG, although serum creatine kinase was within normal range. The chest X-ray film showed that interstitial pneumonia was complicated. She was treated with prednisolone of 60 mg/day. Although erythema and althralgia was improved remarkably, interstitial pneumonia was progressed unfavorably. Therefore pulse therapy with methylprednisolone was administered twice, but failed to respond. After that interstitial pneumonia was progressed rapidly, she was died of respiratory failure. The autopsy findings detected that a degeneration and necrosis of striated muscular fibers in whole body, that a mixed findings of usual interstitial pneumonia and diffuse alveolar damage in the lung. Vasculitis and fiblinous pleuritis was also found in the lung. Malignancy was not detected.
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PMID:[An autopsy case of dermatomyositis with rapidly progressive interstitial pneumonia]. 856

We studied serum concentrations of manganese superoxide dismutase (Mn SOD) and copper-zinc superoxide dismutase (Cu/Zn SOD) in 22 patients with polymyositis and dermatomyositis (PM/DM), 87 patients with four types of muscular dystrophy, 20 patients with amyotrophic lateral sclerosis, and 15 patients with collagen vascular diseases (CVD). Serum levels of Mn SOD were increased only in the patients with PM/DM and CVD, and the elevation was more prominent in those with PM/DM. Levels of Cu/Zn SOD were slightly elevated in some patients with PM/DM and Duchenne muscular dystrophy. In patients with PM/DM, the change in Mn SOD levels corresponded to disease activity as closely as or more closely than those of creatine kinase. The results indicate that serum Mn SOD may be a useful clinical marker for PM/DM.
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PMID:Elevated serum levels of manganese superoxide dismutase in polymyositis and dermatomyositis. 862 97

We report a case of the uncommon association of dermatomyositis and primary biliary cirrhosis in a causasion male of 48-year-old. Diagnosis of dematomyositis was made because of muscle weakness, loss of weight, skin telangiectasia, elevated serum concentration of creatine kinase, polyphasic low amplitudes waves on electromyography and histologic confirmation on muscle biopsy. Diagnosis of primary biliary cirrhosis was made because of elevated values of alcaline phosphatase and gamma glutamyl transferase, elevated values of type II mitochondrial antibody and compatible histological lesions on liver biopsy. We found only ten case reports associating polymyositis and primary biliary cirrhosis. We hypothesize that hepatic and muscle mitochondrial dysfonction may be involved.
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PMID:[Dermatopolymyositis and primary biliary cirrhosis. A rare association]. 865 72

The aim of the current study was to analyze the frequency and characteristics of symptomatic myopathies occurring in rheumatoid arthritis (RA) patients, to correlate these findings with clinical data, and to evaluate their therapeutic implications. All RA patients from a cohort of 350 RA patients from a single institution who developed muscular symptomatology during an 8-year period were included in the study (n = 21). Clinical and laboratory data and electromyographic results were recorded in all cases, and an open muscle biopsy was performed. Weakness and muscle atrophy were the most common symptoms. Serum creatine kinase was increased in 8 cases (38%). Histopathologic study showed type 2 atrophy in 12 cases. In 13 cases, a treatable disease was diagnosed: dermatomyositis (n = 2), d-penicillamine-related dermatomyositis (n = 2), polymyositis (n = 1), muscular mononuclear cell infiltration (n = 3), polyarteritis nodosa (n = 1), glucocorticoid myopathy (n = 3), and toxic chloroquine myopathy (n = 1). In all but 1 patient, muscular clinical response to new therapy and/or drug withdrawal was satisfactory. Although symptomatic muscular involvement in RA is low (6% in the current series), we have found that nearly two thirds of cases were caused by potentially treatable conditions, mainly myositis or toxic myopathies.
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PMID:Muscle involvement in rheumatoid arthritis: clinicopathological study of 21 symptomatic cases. 879 14

This is the first report on idiopathic inflammatory myopathies (IIM) in French Canadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4, 13%), anti-U1RNP (n = 3, 10%), and anti-Ro (n = 2, 7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1 autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.
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PMID:Distinctive features of idiopathic inflammatory myopathies in French Canadians. 887 Jan 12

Inclusion body myositis, polymyositis, and dermatomyositis are three distinct categories of inflammatory myopathy. Some authorities commented on the selective early weakness of the volar forearm muscles, quadriceps, and ankle dorsiflexors in inclusion body myositis. The most important feature distinguishing inclusion body myositis from the other two inflammatory myopathies is the lack of responsiveness to immunosuppressive treatment. Although most patients with inclusion body myositis have characteristic muscle biopsy findings, some cannot be distinguished histologically early from polymyositis. Predicting responsiveness to immunosuppressive medications, independent of muscle histology, would be valuable to clinicians. We retrospectively reviewed the pattern of weakness and other clinical features of 46 patients newly diagnosed with either inclusion body myositis, polymyositis, or dermatomyositis. Asymmetrical muscle weakness with prominent wrist flexor, finger flexor, and knee extensor involvement was specific for inclusion body myositis and unresponsive polymyositis. Male sex, lower creatine kinase levels, slower rate of progression, and peripheral neuropathy were also more common in inclusion body myositis and unresponsive polymyositis than in responsive polymyositis and dermatomyositis patients. Repeat muscle biopsy in 2 patients in the unresponsive polymyositis group demonstrated histological features of inclusion body myositis. We suspect that patients with clinical features of inclusion body myositis but lacking histological confirmation may nonetheless have inclusion body myositis. Our study supports the recently proposed criteria for definite and possible inclusion body myositis.
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PMID:Inclusion body myositis: clinical and pathological boundaries. 887 77

To determine the role of interstitial lung disease (ILD) in the outcome of polymyositis-dermatomyositis (PM/DM), we studied 41 patients who had radiographic evidence of ILD among 51 patients with PM/DM. The patients were divided into 3 groups according to therapy: progressive, no change, or improved. Those classified as progressive had significantly higher rates of skin and lung symptoms, milder muscle symptoms, and lower levels of muscle enzymes than the other 2 groups, and none was positive for the anti-Jo-1 autoantibody. Nine patients with DM died of respiratory failure; they had low serum creatine kinase levels, no anti-Jo-1 antibodies, and a rapidly progressive course before death. Autopsy findings were obtained in 7 patients, and all had diffuse alveolar damage as the main pulmonary histological finding. A subgroup of patients with PM/DM-ILD have a poor prognosis, and ILD in PM/DM is an important prognostic factor.
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PMID:[Outcome in patients with interstitial lung disease and polymyositis-dermatomyositis--a subgroup with poor prognosis]. 895 2

Sight threatening ocular complications are rare in adult patients with dermatomyositis. We encountered a 52-year-old female with dermatomyositis who had severe visual disturbance and rapidly progressive intersitial pneumonia. She was admitted to our hospital because of skin erythema, general fatigue, mild fever, and severe bilateral visual disturbance. Rentinal hemorrhages, cotton wool spots, and macular edema were observed in her fundus at the first ophthalmic examination. A diagnosis of dermatomyositis was made because of the myogenic pattern of her electromyogram, elevation of serum creatine kinase, and skin lesions. Oral prednisolone treatment was started and the retinopathy was improved, but was complicated by acute interstitial pneumonia. The interstitial pneumonia was not respond to steroid pulse therapy with methylprednisolone, and the patient died of respiratory failure on the 47th day after the onset of visual symptoms. In adult dermatomyositis patients, the complication of severe retinopathy should be considered as a risk factor for rapid progress of interstitial pneumonia.
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PMID:[A case of dermatomyositis with severe retinopathy in a patient who died of acute interstitial pneumonia]. 917 Aug 52

We determined serum concentrations of neopterin, soluble tumour necrosis factor (55 kDa) receptor (sTNF-R) and soluble interleukin-2 receptor (sIL-2R) in plasma of 44 patients with polymyositis (PM)/dermatomyositis (DM), including 15 patients with primary PM, 13 patients with primary DM, and 16 patients with myositis and systemic sclerosis in overlap. Concentrations of neopterin, sTNF-R and sIL-2R were measured using commercially available immunoassays. Serum neopterin was increased in 35 of 44 PM/DM patients (80%), sTNF-R in 14 (32%) and sIL-2R in 18 (41%) patients, respectively. There were significant correlations between serum neopterin and sTNF-R, sIL-2R and erythrocyte sedimentation rate (all P < 0.001). Neopterin, as well as sTNF-R and sIL-2R, did not correlate with clinical (neuromuscular and activities of daily living scores) and laboratory (creatine kinase levels) manifestations of myositis. Increased serum levels of neopterin were associated with non-muscular manifestations of PM/DM. In conclusion, serum neopterin appears to be a useful laboratory marker for ongoing immune activation and global disease activity in PM/DM.
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PMID:Elevated serum levels of neopterin in adult patients with polymyositis/dermatomyositis. 923 75


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