Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “DERMATOMYOSITIS”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,135 records · Page 63Linked to original sources
PubMed1995

[Dermatomyositis as a complication of a metastatic melanoma-- case report and review of the literature].

In nearly 60% of men over 50 years of age, dermato-/polymyositis is induced by malignancies. Till now, 9 patients with melanoma-induced dermatomyositis have been reported in the literature. Here we report on a patient suffering from classic pelvic and pectoral girdle proximal weakness, typical heliotrope rash, increased serum CPK values, and biopsy-proven myositis, who died from metastasizing melanoma one year later.

Dermatomyositis↗
PubMed1994

Mineral metabolism in children with dermatomyositis.

OBJECTIVE: To measure calcium metabolism in 12 children with juvenile dermatomyositis (JDM). METHODS: We used dual-tracer stable isotope studies to measure calcium metabolism in 12 children with JDM and a group of 43 healthy children (Group HC) of similar ages. Five of the JDM subjects were receiving steroids (Group JDM-ST) and 7 were not (Group JDM-NS). RESULTS: The rate of calcium absorption in Group JDM-ST was lower than that in Group HC (19 +/- 10% vs 30 +/- 11%, p < 0.05). The lower rate of absorption was associated with a net loss of calcium each day (calculated calcium retention, Vbal, of -35 +/- 14 mg/day compared to +140 +/- 97 mg/day in Group HC, p < 0.01). Group JDM-NS showed slightly lower calcium absorption than Group HC and significantly lower Vbal (+33 +/- 70 mg/day, p < 0.01 vs Group HC) than Group HC. Group JDM-ST subjects also had decreased bone calcium deposition rates. CONCLUSION: Patients with JDM may be at risk for significant loss of bone mineral associated with decreased calcium absorption, especially in the acute phase of their disease when they are receiving steroid therapy.

Absorption↗
PubMed1995

[Cardiac involvement in polymyositis--dermatomyositis associated with Sjogren's syndrome].

A 41 years old woman with polymyositis-dermatomyositis with cardiac involvement is presented. The patient evolved with congestive heart failure, the electrocardiogram showed a left anterior hemiblock, lack of progression of R waves from V1 to V4 and unspecific ST and T alterations. Echocardiogram and cardiac catheterization showed global ventricular disfunction and pulmonary hypertension. An endomyocardial biopsy performed at the apex of the right ventricle showed mononuclear inflammatory infiltration, myocardial fiber degeneration and fibrosis. Initially, the patient responded well to diuretic, vasodilator and steroid therapy. Posteriorly she developed an atrial flutter that required electrical cardioversion and later died suddenly during the course of an acute pneumonia.

Adult↗
PubMed1994

[A case of organic psychosis in dermatomyositis. Etiopathogenetic hypothesis].

The authors describe a girl affected by dermatomyositis, with a quick and grave evolution, which required urgent tracheostomy to reduce the dysphagic symptoms. During the observation the patient showed psychotic symptoms. After the revision of literature, in which no such complication has ever been described, the authors propose an etiopathogenetic interpretation for a generalized acute psycho-organic syndrome, supposing a cerebral hypoxic damage, as vasculitic manifestation, probably complicated by symptoms of hysterical conversion, due to the isolation of the patient in the Department of Intensive Therapy and to the severe physical conditions.

Adolescent↗
PubMed1995

von Willebrand factor in juvenile dermatomyositis.

OBJECTIVE: To evaluate whether plasma von Willebrand factor (vWF) levels are a useful indicator of disease activity in juvenile dermatomyositis (DM). METHODS: Serum vWF levels were prospectively measured in 15 patients with juvenile DM and were compared to serum muscle enzyme levels, muscle strength, and presence of extramuscular manifestations. RESULTS: 6/15 active disease periods were accompanied by an increase in vWF; 9 were not. Elevated vWF levels did not relate to the presence of active skin disease or calcinosis. vWF was not consistently related to muscle strength, CPK, or aldolase in the study group. Two patients had elevations of vWF in association with viral infections while their DM was quiescent. CONCLUSION: An elevated vWF level is often indicative of a disease exacerbation in DM, and thus may be helpful in managing difficult cases. However, it is not consistently elevated in, nor specific for active disease in DM. It therefore cannot be recommended as a routine test in patients with DM. When used, results should be interpreted with caution.

Adolescent↗
PubMed1995

Measuring disability in juvenile dermatomyositis: validity of the childhood health assessment questionnaire.

OBJECTIVE: Physical disability is perhaps the most important outcome of juvenile dermatomyositis (JDM). No functional assessment tool has been validated for inflammatory myopathies either in children or adults. We studied the measurement properties of the Childhood Health Assessment Questionnaire (CHAQ) in children with JDM. METHODS: We studied 37 patients followed at the JDM clinic and compared the results obtained by the CHAQ to a global disease severity score and quantitative muscle strength testing measured by sphygmomanometry (construct validity). We also measured the reliability of the CHAQ and its responsiveness to clinical change. RESULTS: For the initial measurement of each subject, the correlation between disease severity and CHAQ was high [Spearman's correlation, (rs = 0.71, p < 0.002)]. Disability as measured by the CHAQ was inversely correlated with proximal muscle strength (hip abduction rs = -0.57, p < 0.002; shoulder abduction rs = -0.51, p < 0.01) but, as expected, less so with more distal muscle strength (knee extension rs = -0.40, p = 0.05; grip strength rs = -0.079, p > 0.20). The CHAQ was reliable in subjects who showed no clinical change in muscle strength (intraclass correlation coefficient = 0.87) and responsive to treatment induced clinical change (responsiveness coefficient = 0.90). CONCLUSION: The CHAQ can serve as a valid and sensitive tool in the evaluation of functional outcomes in JDM.

Adolescent↗
PubMed1995

Muscle strength assessment in polymyositis and dermatomyositis evaluation of the reliability and clinical use of a new, quantitative, easily applicable method.

OBJECTIVE: To evaluate the feasibility and the reliability of a quantitative method of maximal isometric strength measurement for the assessment of patients with myositis in clinical practice and research. METHODS: Three observers independently examined the strength of 13 muscle groups of 7 patients with stable polymyositis and dermatomyositis (PM/DM) using a handheld pull-gauge. Reliability was assessed following a balanced random 3-way cross classification with interactions and using the analysis of variance method. Serial followup strength data for 2 patients were compared to change in creatinine kinase (CK) levels. RESULTS: All measurements could be performed without causing pain to the patients. One single test including 13 muscle groups was done in 7 min or less. Both intra and interobserver correlations were strong and significant for all muscle groups. The intraobserver reliability ranged from 0.88 for elbow extensors to 0.98 for knee extensors and cervical spine flexors. The interobserver reliability ranged from 0.81 for elbow flexors to 0.98 for knee extensors and cervical spine flexors. The followup of 2 cases yielded moderate to strong correlations of serial strength measurements with CK levels. CONCLUSION: Serial measurement of isometric muscle strength with a handheld pull-gauge is a feasible, inexpensive, time efficient and reliable method and may provide additional quantitative information in the clinical assessment of patients with myositis.

Adult↗
PubMed1995

Classification criteria for polymyositis and dermatomyositis.

OBJECTIVE: The establishment of classification criteria for polymyositis (PM) and dermatomyositis (DM). METHODS: Questionnaires inquiring about patients with DM, PM, systemic lupus erythematosus, progressive systemic sclerosis and noninflammatory neuromuscular diseases were distributed to the main medical institutes in Japan. Data were collected and analyzed by computer. RESULTS: Among skin lesions of DM, heliotrope rash, Gottron's sign and erythema or purpura on the extensor surfaces of the extremity joints were shown to be distinguishing criteria. In both DM and PM, proximal muscle weakness, muscle grasping and spontaneous pain, nondestructive arthritis or arthralgia, elevated CK or aldolase level, presence of systemic inflammatory signs, myogenic changes on EMG, positive and anti Jo-1 antibody and pathologic findings compatible with inflammatory myositis were distinguishing criteria items. CONCLUSION: When a patient satisfies one of 3 skin lesion items and at least 4 other items, he or she shall be classified as having DM, sensitivity 94.1%. When a patient satisfies at least 4 items other than skin lesion items, he or she shall be classified as having PM, sensitivity 98.9%. Specificity of DM and PM is 95.2%.

Dermatomyositis↗
PubMed1994

Idiopathic inflammatory myopathies: inclusion-body myositis, polymyositis, and dermatomyositis.

In this review, the main emphasis is on new advances concerning sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Polymyositis and dermatomyositis are reviewed briefly. Hypotheses are presented regarding the possible cause and significance of abnormally accumulated beta-amyloid protein, two other epitopes of beta-amyloid precursor protein, hyperphosphorylated tau, alpha 1-antichymotrypsin, ubiquitin, and prion protein in sporadic inclusion-body myositis and hereditary inclusion-body myopathy. Because most of those proteins are also accumulated at the neuromuscular junction, "junctionalization" of other muscle fiber nuclei is a possibility. Attention is given to the fact that vacuolated muscle fibers in hereditary inclusion-body myopathy may represent early changes because they are virtually free of congophilic amyloid deposit but, like sporadic inclusion-body myositis, contain large accumulations of beta-amyloid protein and prion.

Aged↗
PubMed1994

The overall and temporal association of cancer with polymyositis and dermatomyositis.

OBJECTIVE: To summarize the association of cancer with adult polymyositis (PM) and dermatomyositis (DM) and to test whether myositis precedes or follows cancer diagnosis, with the latter suggesting that myositis is paraneoplastic. METHODS: Using primary patient data, we performed an analysis of all published case control and cohort studies evaluating the association of myositis and malignancy. We evaluated the overall association of cancer within 10 years of myositis diagnosis and separately assessed cancer occurrence in periods 5 years before and 5 years after myositis diagnosis. RESULTS: Four case control or cohort studies met inclusion criteria providing 1078 myositis cases (565 PM and 513 DM) with a comparable number of controls. One hundred fifty-three myositis patients had cancer within 10 years of diagnosis. Despite reports of no myositis association with cancer, each study's data suggested an association of both PM and DM with cancer. Overall odds ratio (OR) for the association of cancer with DM = 4.4 (9.5% CI 3.0, 6.6) and for PM, OR = 2.1 (95% CI 1.4, 3.3). Cancer risk was high both before and after DM diagnosis, but for PM, cancer risk was increased only after myositis diagnosis. CONCLUSION: DM is associated with cancer, and our results suggest that DM is often paraneoplastic. The high rate of malignancy only after PM diagnosis is consistent with cancer detection bias, but may suggest joint susceptibility of affected individuals to both cancer and myositis.

Case-Control Studies↗
PubMed1994

Relationship of cancer to inflammatory muscle diseases. Dermatomyositis, polymyositis, and inclusion body myositis.

Dermatomyositis (DM) is probably associated with a greater frequency of malignancy than expected in the general population. For polymyositis (PM), there does not appear to be greatly increased risks. Ovarian cancer may be over-represented in women with DM. A paraneoplastic course occurs in some patients with DM, but is unusual. Even young patients with DM should be evaluated. The malignancy evaluation should be directed by symptoms, findings on physical examination or laboratory testing, or be age-appropriate.

Adult↗
PubMedDate not supplied

Treatment of polymyositis and dermatomyositis.

Polymyositis and dermatomyositis are serious inflammatory muscle disorders which may present life-threatening complications. It is important to recognise and treat the condition in the early stages of the disease. Corticosteroids remain the mainstay of treatment, and their resistance and other forms of immunotherapy are discussed.

Adrenal Cortex Hormones↗
PubMed1994

[Dermatomyositis and Wilson disease].

A 15 year-old girl developed both a dermatomyositis and a Wilson's disease. A clinical remission was obtained with steroids and D-penicillamine. The potential role of cupric intoxication in the pathogeny of the muscular syndrome is discussed.

Adolescent↗
PubMed1994

Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients.

OBJECTIVE: Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many possible interventions are available to treat these patients: corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. But these therapies are not always effective and may be responsible for certain serious side effects. Polyvalent intravenous immunoglobulin (IVIG) has been tried with success in inflammatory myopathies after failure of traditional treatment. An attempt was made to evaluate the efficacy of IVIG as first line therapy in patients with PM or DM. METHODS: Eleven Caucasian patients [6 women, 5 men, mean age 55.6 (SD 10.1) years], with active recent inflammatory myopathy, were treated by high doses of IVIG as first choice. The average duration of inflammatory myopathy before IVIG was 9.6 months (SD 10.2 months, with a range of 1 month to 3 years). Five patients had PM and 6 had DM. None had myositis associated with connective tissue disease. Two patients had a history of malignant disease: 1 lymphoma and 1 breast tumor with relapse of the malignancy during the study. One patient had a probable lung carcinoma and in another patient, ovarian carcinoma was diagnosed a few months after the onset of IVIG. We used preparations of polyvalent human i.v. gamma globulins with intact IgG. All patients received 1 g/kg daily for 2 days each month. The mean course of treatment was 4 months. RESULTS: Clinical assessment, evaluated by proximal muscle power and biochemical tests, was carried out before each treatment period. Significant clinical improvement was noted in only 3 of the 11 patients (one with acute coxsackie virus B infection, and one with possible drug induced myopathy). Mean muscle power estimated for the 11 patients before and after IVIG therapy was not significantly improved. Eight patients showed significant biochemical improvement (more than 50%). Mean CK levels for the 11 patients showed a statistically significant decrease during IVIG therapy (p < 0.01). Minor IVIG side effects were noted in one patient. CONCLUSION: IVIG therapy seems effective rarely as first therapy in patients with inflammatory myopathy but may be considered especially in viral or drug induced myopathy. IVIG therapy as the first treatment may also be tried in patients with contraindication for steroids, and in mild myopathy, especially in the elderly, to avoid steroid induced side effects.

Adult↗
PubMed1994

[Scleroderma, dermatomyositis].

Systemic sclerosis (scleroderma, SSc) is a progressive and often fatal disorder characterized clinically by sclerotic changes in the skin, joints and internal organ systems such as the gastrointestinal tract, heart, lungs and kidneys, moreover pathologically by abnormalities of mucopolysaccharides, fibrous tissue deposition, atrophy of parenchymal structures in skin and various internal organs, and by vascular insufficiency. Little is known of its etiology and pathogenesis. Transforming growth factor-beta (TGF-beta), platelet derived growth factor-AA (PDGF-AA) and PDGF-alpha receptor interaction may play an important role in the pathogenesis of scleroderma. Furthermore, many of the proteoglycans act as modulators of growth factor activities. Dermatomyositis is also a complex connective tissue disease of unknown etiology, in which inflammatory change in the skin, muscle, and lung in association with vascular insufficiency and internal malignancy.

Blood Vessels↗
PubMedDate not supplied

Juvenile dermatomyositis: serial studies of circulating autoantibodies to a 56kD nuclear protein.

In this study we report that circulating antibodies recognising a 56kD protein, which is a component of large nuclear ribonuclear particles, are commonly found in children with juvenile onset dermatomyositis (JDM). These autoantibodies, as detected by Western blotting, were present in over 90% (24/26) of sera from JDM patients, which exceeds the number of patients with adult onset myositis who express this antibody (up to 85%). In addition, they were not found in healthy controls. Serial bleeds taken during the course of the disease in eleven children with JDM enabled us to follow the titre of anti-56kD autoantibodies. Sera were also tested by indirect immunofluorescence for anti-nuclear antibodies (ANA) using Hep2 cells as substrate. These studies revealed two distinct patient groups: Group 1 with anti-56kD antibody positive and ANA positive; and Group 2 with anti-56kD antibody positive and ANA negative. In Group 1 there was some correlation between disease activity and anti-56kD levels which was absent among patients in Group 2.

Antibodies, Antinuclear↗
PubMedDate not supplied

Juvenile dermatomyositis (JDMS): new clues to diagnosis and pathogenesis.

The localization of focal inflammatory myopathy (IM) is aided by MRI, which facilitates diagnostic testing (muscle biopsy or EMG). Antibodies to antigens involved in protein synthesis are specific for IM and characterize distinct subsets of adult IM, but are rare in pediatric IM. In definite juvenile dermatomyositis, serological indicators of disease activity include neopterin and von Willebrand factor antigen, markers of macrophage activation, and endothelial cell damage, respectively; nailfold capillarioscopy documents small blood vessel involvement. Monitoring the percentage of circulating B cells may indicate the response to therapy.

B-Lymphocytes↗
PubMed1994

Strength in polymyositis and dermatomyositis: best outcome in patients treated early.

OBJECTIVE: To study the influence of clinical variables on improvement in muscle strength in patients with polymyositis and dermatomyositis. METHODS: We biomechanically examined muscle strength over the course of 2.54 years of 65 patients. RESULTS: Disease duration before therapy was predictive of outcome. Those treated within 6 months or one year of disease onset had better outcomes than those with later treatment. In addition, among those who gained strength, degree of elevation of creatine kinase was correlated with greater gains. CONCLUSION: Early treatment after disease onset increases the chance of a favorable response.

Biomechanical Phenomena↗