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)

Amyopathic dermatomyositis (ADM) is characterized by the typical cutaneous features of dermatomyositis and minor involvement of the skeletal muscles. A 50-year-old woman had fever, reddening and pain in the distal part of all four limbs, and cutaneous findings such as Gottron's papules and periorbital heliotrope. She showed no muscle weakness or atrophy, and her serum creatine kinase was within the normal range. Electromyography showed no myopathic pattern. Magnetic resonance imaging (MRI) recorded abnormal hyperintensity in the fascia and muscle of the tibialis anterior. A biopsy from the tibialis anterior muscle showed fasciitis and mild myopathic changes with focal perivascular infiltration. This patient also presented with interstitial pneumonitis, although evaluation for malignancy was negative. With steroid therapy, her symptoms and MRI abnormality disappeared within 2 months. This case is therefore considered to be a variant of ADM, presenting as dermato-fasciitis.
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PMID:A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis. 1504 32

Because the clinical significance of von Willebrand factor (vWF), a marker of endothelial injury, has not been well studied in adult patients with dermatomyositis (DM), we evaluated whether plasma vWF levels are useful as an index of disease activity in these patients. We measured plasma vWF antigen levels in 11 patients with active adult DM, 13 patients with inactive DM, and 18 healthy subjects using an enzyme-linked immunosorbent assay. The association of vWF level with clinical condition and muscle-derived enzyme leakage among DM patients was examined using analysis of covariance and logistic regression analysis. Furthermore, we studied the effects of treatment on the vWF antigen level. The mean vWF antigen level was significantly higher in active DM patients than in inactive DM patients and healthy subjects. Higher vWF levels were associated with clinical symptoms, such as general fatigue, fever, and muscle weakness. They were also associated with the levels of aspartate aminotransferase, alanine aminotransferase, and aldolase, but not with those of lactate dehydrogenase and creatine kinase (CK). vWF antigen was correlated with muscle enzymes except for CK. The plasma vWF levels in six patients with active DM significantly decreased after successful corticosteroid treatment. Plasma vWF level may be considered a useful marker of disease activity in adult DM patients.
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PMID:Clinical significance of von Willebrand factor in patients with adult dermatomyositis. 1556 94

A 78 year-old woman was admitted to our hospital because of subacutely progressive dysarthria, dysphagia, proximally dominant muscle weakness and erythema in the neck and back. She was diagnosed as having rheumatoid arthritis (RA) at the age of 60 and treated with bucillamine (BUC) for 8 years. Laboratory tests included a rheumatoid factor of 1,472U/ml. Serum creatine kinase level was slightly elevated. The activated T cells in the peripheral blood were markedly increased. Needle EMG demonstrated myogenic changes. The magnetic resonance image of the left upper arm showed diffuse muscle atrophy and inflammatory changes in the triceps muscle. The muscle biopsy revealed perivascular inflammatory cell infiltraton and type II fiber atrophy. A biopsy from the skin showed mild perivascular inflammatory cell infiltraton. According to the results of these findings, she was thought to have dermatomyositis due to BUC. After withdrawal of BUC followed by the administration of prednisolone 1mg/kg, her symptoms improved and activated T cells in the peripheral blood were decreased. In Japan, BUC is widely accepted as an effective drug in the treatment of RA, even though it is known to induce some autoimmune diseases. However, the mechanism of the development of autoimmune disease is unclear. We considered that the long-term use of bucillamine could trigger an autoimmune response such as an increase in activated T cells and the development of dermatomyositis-like clinical features in our patient. In conclusion, when RA patients treated with BUC show a clinical picture compatible with dermatomyositis, its causative relationship has to be considered.
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PMID:[Bucillamine-induced dermatomyositis-like clinical features in a patient with rheumatoid arthritis]. 1571 1

We report a case of corticosteroid-resistant nonspecific interstitial pneumonia associated with dermatomyositis. The patient was successfully treated with a combination of cyclosporin A (CsA) and prednisolone. A 28-year-old man was admitted complaining of skin eruptions, myalgia, and weakness, and dyspnea on exertion. Characteristic skin lesions such as erythema around the nails, teleangiectasis, and edema of the eyelids were observed. Dermatomyositis was diagnosed based on the diagnostic criteria, including the skin biopsy findings. Laboratory examination showed elevation of creatine kinase, but the anti-Jo-1 antibody was negative. Chest X-ray on admission showed reticular shadows and airspace consolidation mainly in both lower lung fields. Lung biopsy specimens taken under video-assisted thoracoscopic surgery revealed infiltration of lymphocytes into the thickened alveolar septa, and this revelation was compatible with the description of cellular nonspecific interstitial pneumonia. Administration of corticosteroids with steroid pulse therapy resulted in with improvement of interstitial pneumonia. However, after the combination therapy of corticosteroid with daily oral administration of 100-150 mg of CsA had been initiated, hypoxemia recovered swiftly in 5 days, and respiratory symptoms improved gradually, but steadily. Oral administration of corticosteroid and CsA was eventually tapered off and terminated completely 5 years later. The patient has been quite well with no treatment for two and a half years. The case presented here implies that CsA combined with corticosteroid may be efficacious in the treatment of interstitial pneumonia associated with dermatomyositis which is refractory to corticosteroid therapy.
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PMID:[A case of corticosteroid-resistant nonspecific interstitial pneumonia associated with dermatomyositis successfully treated with cyclosporin A]. 1580 Dec 87

The objective of the current report was to determine the relapse rates and characterize the nature of relapses during the disease course of adult patients with idiopathic inflammatory myopathies (IIM). A retrospective cohort study of 53 medical records of patients with polymyositis (PM), dermatomyositis (DM), connective tissue disease (CTD)-associated myositis, and malignancy-associated myositis at an academic rheumatology center was performed. Medical records were reviewed to determine clinical presentation, initial treatment, and clinical follow-up, with an emphasis on relapses. Relapses were defined as a sustained elevation in serum creatine kinase (CK) levels in the absence of an alternative etiology. Patients were followed for an average of 65+/-43 months. All patients received corticosteroids, and 35 patients received additional immunosuppressive medications as part of their initial treatment. Serum CK levels normalized in 51 patients, and muscle strength normalized in 43 patients. Biochemical relapse was observed in 33 patients (65%). Patients with PM and CTD-associated myositis had a higher relapse rate compared to DM and malignancy-associated myositis patients. Multiple relapses were observed in 17 patients. Relapses tended to occur within the first 2 years after treatment initiation and during the tapering phase of treatment. No risk factors were unequivocally identified, although advanced age and increased duration of symptoms prior to treatment initiation had nonsignificant associations with increased risk of relapse. In conclusion, initial treatment of IIM results in a high rate of normalization of serum CK and muscle weakness. However, physicians should be aware of the high rate of relapse in patients with IIM.
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PMID:Characterization of relapses in adult idiopathic inflammatory myopathies. 1626 4

Dermatomyositis is an inflammatory myopathy characterized by muscle weakness and inflammation. In contrast to polymyositis and inclusion body myositis, humoral immune mechanisms appear to contribute to the pathogenesis of dermatomyositis. A 56-year-old man with dermatomyositis resistant to conventional therapies was treated with 6 weekly infusions of the anti-CD-20 monoclonal antibody, rituximab, at a dosage of 100 mg/m in addition to other agents. The patient demonstrated a remarkable clinical response as indicated by an increase in muscle strength and a decline in creatine kinase enzymes. B-cell depletion therapy with rituximab used alone or in combination with other immunosuppressive therapies may be a viable option in patients with dermatomyositis as well as other autoimmune diseases refractory to current therapies.
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PMID:Rituximab in the treatment of refractory dermatomyositis. 1635 73

The objective of this study was to assess the long-term outcome of polymyositis (PM) and dermatomyositis (DM) and the factors predictive of this outcome in a nationwide series in Finland. One hundred and seventy-six patients with PM and 72 patients with DM diagnosed in Finland in 1969-1985 were selected from the national hospital discharge register according to the diagnostic criteria of Bohan and Peter and followed up until death or till the end of August 1995. Gender, age, delay of therapy, serum creatine kinase, erythrocyte sedimentation rate, initial dose of corticosteroids and duration of cytostatic therapy were assessed as factors prognostic of death. The 5-year survival rate for PM was 75% [95% confidence interval (CI): 68-81%] and that for DM 63% (50-73%), and the respective 10-year survival rates were 55% (47-62%) and 53% (41-64%). The median survival for polymyositis was 11.0 years (95% CI: 9.5-13.3) and that for DM 12.3 years (5.5-20.7). The standardized mortality ratio for the combined group of PM and DM showed approximately threefold mortality compared to the general population. Compared to PM patients, the subjects with DM had a 1.47-fold (95% CI: 0.99-2.12) age- and sex-adjusted risk of mortality. Except for age in both groups and the delay in diagnosis in the PM group, no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 2.16 for death (95% CI: 0.95-4.50) in the DM group and 1.99 (95% CI: 1.01-3.94) in the PM group. A comparison of the causes of death in the PM and DM groups showed that the patients with DM had a greater risk of dying from cancer (age-adjusted HR 5.11, 95% CI: 2.31-11.3). According to this nationwide analysis of survival and its prognostic factors in patients with PM and DM, the latter group had an increased age-adjusted risk for mortality compared to the former. The difference seems to be mostly explained by the fact that the patients with DM had a higher risk of dying from cancer.
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PMID:Prognosis and mortality of polymyositis and dermatomyositis patients. 1647 98

Dermatomyositis is a rare inflammatory myopathy that has characteristic cutaneous lesions. Although many malignancies are associated with dermatomyositis, urogenital malignancies have rarely been reported to be associated with dermatomyositis. We report here on the first case of ureteral cancer associated with dermatomyositis. A 42-year-old man presented to us with a skin rash. A clinical diagnosis of dermatomyositis was made due to the skin lesions, muscle weakness, arthralgia, the increased erythrocyte sedimentation rate and the increased creatine kinase level. The patient revealed microscopic hematuria and abnormal urine cytology during the investigation for the underlying malignancy. Retrograde pyelography demonstrated a suspicious lesion in the right mid-ureter, and the ureteroscopic biopsy revealed the urothelial carcinoma. Although an operation was recommended, the patient died of pneumonia associated with his interstitial lung disease, which is one of the poor prognostic indicators of dermatomyositis.
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PMID:Ureteral cancer associated with dermatomyositis. 1673 68

The aim of this study was to assess the prevalence and the common type of malignancies in Korean patients with polymyositis (PM) and dermatomyositis (DM) and to evaluate the differences of clinical and laboratory findings between patients with malignancy and those without malignancy. Forty-one Korean patients, who were diagnosed as PM or DM, were enrolled in this study. They fulfilled the Bohan and Peter's criteria for a definite diagnosis of PM and DM. Patients with PM were 25 and those with DM were 16. Eleven out of 41 patients (26.8%) had malignancies. The malignancy was diagnosed simultaneously or later in 81.8% of patients with inflammatory myopathy (IM). The breast cancer was the most common malignancy. In this study, forty three years old as a screening age for malignancy had 88.9% sensitivity and 50.2% specificity. The serum levels of creatine kinase (CK) were significantly lower in patients with malignancy than those without malignancy.
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PMID:Malignancies in Korean patients with inflammatory myopathy. 1694 42

A 56-year-old Japanese man was admitted to our hospital due to a fever of unknown origin. He had had a history of extranodal natural killer (NK)/T-cell lymphoma, nasal type, and had been in complete remission for 7 years until June 2003, when he developed high fever, eyelid swelling, and muscular weakness. Serum creatine kinase levels were elevated. Histopathological examination of skin and muscle biopsy specimens revealed subcutaneous infiltration of lymphoid cells positive for CD3, CD56, and Epstein-Barr virus-encoded small nuclear RNA-1. We report this unique case of Epstein-Barr virus-associated lymphoma mimicking dermatomyositis.
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PMID:A case of Epstein-Barr virus-associated natural killer/T-cell lymphoma presenting as dermatomyositis: extranodal relapse after 7 years in remission. 1702 9


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