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

We report a case of dermatomyositis associated with prostatic carcinoma. A 69-year-old male was admitted to the Department of Internal Medicine with the chief complaint of general fatigue, appetite loss and facial anthema. Abdominal ultrasound demonstrated swollen periaortic lymph nodes and the margin of prostate was unclear. Prostatic carcinoma was suspected based on digital rectal examination, so he was admitted to our department. Serum prostate specific antigen level was 190 ng/ml. He was examined by a dermatologist because of deterioration of anthema. Dermatomyocitis was demonstrated by dermatoses (edema erythema at face, neck and limbs, nail fold thrombosis and poikiloderma), high serum level of creatine phosphokinase and a decrease in muscular strength (especially at the proximal musculus). There was no interstitial pneumonitis or malignancy of the digestive system. On needle biopsy of the prostate and quadriceps femoris muscle, prostatic carcinoma (poorly differentiated adenocarcinoma, Gleason score 5 + 5) and myositis were suspected. The stage of prostatic carcinoma was T4N1M1. The patient was treated by administration of diethylstilbestrol phosphate and prednisolone for prostatic carcinoma and dermatomyositis, respectively, but he died of multiple metastasis of the tumor 1 year and 5 months later. Dermatomyocitis is associated with malignancy more frequently than any other collagen disease. In Japan, it is frequently complicated by gastric, lung and mammory cancers, but rarely by prostatic carcinoma. To our knowledge, this is the fourth case of prostatic carcinoma associated with dermatomyocitis in Japan.
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PMID:[A case of dermatomyositis associated with prostatic carcinoma: a case report]. 1510 Nov 64

A 61-year-old man was admitted to our hospital because of edematous erythema on his upper eyelids and dry cough. No subjective nor objective findings suggestive of skeletal muscle involvement, such as muscle weakness and elevated levels of aldolase and creatine phosphokinase were noted. Chest high-resolution computed tomography revealed a ground glass opacity and consolidation of his lower lung. Skin biopsy findings were compatible with dermatomyositis. Therefore, he was diagnosed as amyopathic dermatomyositis (ADM) with acute interstitial pneumonia and treatment with steroid pulse therapy was started. Since histological evaluation showed diffuse alveolar damage during the initial treatment, the treatment was changed into the combination therapy of prednisolone and cyclosporine. However, his acute interstitial pneumonia did not respond to this treatment and passed away by aggravation of a breathing state and concurrence of disseminated intravascular coagulation. Japanese patients with ADM have been shown to be more frequently associated with intractable acute interstitial pneumonia than Caucasian patients, suggesting that the racial difference influences the occurrence of acute interstitial pneumonia in ADM. Since autoantibodies specific for ADM have not been detected, we performed immunoprecipitation analysis using 35S methionine-labeled K562 cells to identify them. His sera immunoprecipitated a polypeptide of 140 kDa. The 140 kDa polypeptide might be one of autoantibodies specific for ADM with acute interstitial pneumonia, although future analysis using a larger number of patients with ADM will be required to confirm this result.
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PMID:[A case of amyopathic dermatomyositis with acute interstitial pneumonia (DAD pattern)]. 1516 31

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

We describe response to rituximab treatment of refractory inflammatory myopathy. Three patients with long-standing polymyositis (PM) or dermatomyositis (DM) poorly responsive to prednisone combined with several immunosuppressants were given intravenous rituximab 1,000 mg on Days 0 and 14. Prior to rituximab, each had significant proximal weakness with creatine phosphokinase (CPK) elevation to>3 times the normal upper limit (range 789-3,123 U/l). Patients were receiving prednisone plus methotrexate (MTX) or azathioprine. CPK decrease was observed 1 month post-infusion, with normalization of levels averaging 4.6 months (range 2.6-7.7 mo). Muscle strength improved in all, with strength returning to normal in 2. Average daily prednisone dose decreased from 16.7 mg (range 10-20 mg) to 4 mg (range 0-7 mg) after infusion. MTX dose was tapered by 50% in 2 patients. The third patient eventually discontinued all additional therapies. Percentage of CD19+ cells in each were suppressed at 0-1% 5 to 6 months after infusion (normal 5-21%). Elevated CPK with return of clinical symptoms occurred in 2 patients 6 and 10 months post-infusion, requiring rituximab retreatment. CD19+ cells remained suppressed at 1% in one patient, but were almost normal at 4% in the other. The third patient remains disease-free 12 months after initial treatment, even though her CD19+ cells are now normal at 8%. Thus, short-term beneficial effects with rituximab were observed in patients with DM and PM. However, the need for retreatment did not correlate with levels of CD19+ cells.
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PMID:Rituximab as therapy for refractory polymyositis and dermatomyositis. 1654 75


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