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 667 records · Page 37Linked to original sources
PubMed2006

Study of subcutaneous fat in children with juvenile dermatomyositis.

OBJECTIVE: Lipodystrophy is a recently recognized complication of juvenile dermatomyositis (juvenile DM). Until now, the diagnosis has been based only on the physical appearance of the patient. We quantified the patterns of fat distribution in a cohort of patients with juvenile DM. METHODS: Twenty patients with juvenile DM were enrolled along with a matching number of controls. Both groups underwent standard anthropometric measurements including assessment of skinfold thickness using Harpenden Skinfold Caliper (Holtain, Dyfed, UK). Glucose tolerance test and serum lipid estimates were performed in the study group but not in controls. RESULTS: Patients with juvenile DM had lower mean weight, height, and mid-upper-arm circumference as compared with controls; these differences were statistically significant (P < or = 0.05). Eight (40%) of 20 patients had lipodystrophy on physical appearance. When assessed by skinfold caliper, there was loss of subcutaneous fat at the mid-axillary site in 65% of patients, at the subscapular in 60%, and at the suprailiac site in 55%. Serum triglyceride levels were increased in 12 of the 18 patients who underwent this test. Oral glucose tolerance test results were normal in all 20 patients. CONCLUSION: Sixty-five percent of our patients with juvenile DM were found to have loss of subcutaneous fat on quantification compared with 40% on physical appearance alone. Maximum fat loss occurred at the mid-axillary skinfold site. A significant number of patients with juvenile DM (66%) had hypertriglyceridemia. We hypothesize that lipodystrophy and hypertriglyceridemia could well be integral components of what may be an expanded juvenile DM syndrome.

Adipose Tissue↗
PubMed2006

Composition of calcifications in children with juvenile dermatomyositis: association with chronic cutaneous inflammation.

OBJECTIVE: Calcific deposits develop in 20-40% of children with juvenile dermatomyositis (juvenile DM), contributing to disease morbidity and mortality. This study was undertaken to define the structure and composition of these deposits and to characterize their association with chronic inflammation. METHODS: We examined calcific deposits from 5 children with juvenile DM (2 boys and 3 girls). The crystal structure and mineral content of the deposits was analyzed by x-ray diffraction, Fourier transform infrared spectroscopy, and imaging. The protein content of the deposits, following solubilization, was assayed by Western blotting. RESULTS: All 5 children had both a young age at disease onset (mean +/- SD 3.3 +/- 1.9 years) and, despite therapy, persistent cutaneous inflammation (mean +/- SD duration 81.3 +/- 58.7 months). The bone proteins, osteopontin, osteonectin, and bone sialoprotein, were identified in the protein extracts; the only mineral detected was hydroxyapatite, but the tissue was distinct from bone, with an extremely high mineral content and an irregular distribution of mineral. CONCLUSION: These results indicate that chronic cutaneous inflammation may contribute to the formation of hydroxyapatite-containing pathologic calcifications in children with juvenile DM.

Adolescent↗
PubMed2006

A novel autoantibody to a 155-kd protein is associated with dermatomyositis.

OBJECTIVE: In polymyositis and dermatomyositis (DM), identified autoantibodies occur in <50% of adult patients and in a smaller proportion of children. This study was undertaken as part of a larger effort to define novel autoantibodies that assist in the clinical evaluation of myositis. METHODS: Sera from children and adults satisfying criteria for idiopathic inflammatory myopathies and from patients with other connective tissue diseases (CTDs), patients with noninflammatory myopathies, and healthy individuals were tested for autoantibodies by immunoprecipitation (IP). A previously unrecognized autoantibody that immunoprecipitated a 155-kd protein along with a weaker 140-kd protein was seen. When the presence of this anti-p155 autoantibody in test sera was suggested based on IP results, it was confirmed by immunoblotting of immunoprecipitates. RESULTS: Sera from 51 of 244 myositis patients (21%), including 30 with juvenile DM (29%), 5 with juvenile CTD-associated myositis (33%), 8 with adult DM (21%), 6 with cancer-associated DM (75%), and 2 with adult CTD-associated myositis (15%), were found to have anti-p155 autoantibody. One of 49 patients with lupus, and none of 89 others without myositis, had anti-p155. Caucasian patients with anti-p155 had a unique HLA risk factor, DQA1*0301 (odds ratio 5.4, corrected P = 0.004). In adults with anti-p155, of several clinical features assessed only the frequency of V-sign rash was increased, but patients with this antibody were clinically distinct from those with autoantibodies to aminoacyl-transfer RNA synthetases. CONCLUSION: A newly recognized autoantibody, anti-p155, is associated with DM and cancer-associated DM, and is one of the most common autoantibodies in this condition, occurring as frequently in children as in adults. The clinical features and immunogenetics associated with anti-p155 differ from those associated with antisynthetases.

Adult↗
PubMed2006

Immunogenetic risk and protective factors for juvenile dermatomyositis in Caucasians.

OBJECTIVE: To define the relative importance (RI) of class II major histocompatibility complex (MHC) alleles and peptide binding motifs as risk or protective factors for juvenile dermatomyositis (DM), and to compare these with HLA associations in adult DM. METHODS: DRB1 and DQA1 typing was performed in 142 Caucasian patients with juvenile DM, and the results were compared with HLA typing data from 193 patients with adult DM and 797 race-matched controls. Random Forests classification and multiple logistic regression were used to assess the RI of the HLA associations. RESULTS: The HLA-DRB1*0301 allele was a primary risk factor (odds ratio [OR] 3.9), while DQA1*0301 (OR 2.8), DQA1*0501 (OR 2.1), and homozygosity for DQA1*0501 (OR 3.2) were additional risk factors for juvenile DM. These risk factors were not present in patients with adult DM without defined autoantibodies. DQA1 alleles *0201 (OR 0.37), *0101 (OR 0.38), and *0102 (OR 0.51) were identified as novel protective factors for juvenile DM, the latter 2 also being protective factors in adult DM. The peptide binding motif DRB1 (9)EYSTS(13) was a risk factor, and DQA1 motifs F(25), S(26), and (45)(V/A)W(R/K)(47) were protective. Random Forests classification analysis revealed that among the identified risk factors for juvenile DM, DRB1*0301 had a higher RI (100%) than DQA1*0301 (RI 57%), DQA1*0501 (RI 42%), or the peptide binding motifs. In a logistic regression model, DRB1*0301 and DQA1*0201 were the strongest risk and protective factors, respectively, for juvenile DM. CONCLUSION: DRB1*0301 is ranked higher in RI than DQA1*0501 as a risk factor for juvenile DM. DQA1*0301 is a newly identified HLA risk factor for juvenile DM, while 3 of the DQA1 alleles studied are newly identified protective factors for juvenile DM.

Adolescent↗
PubMed1992

Pericardial tamponade in juvenile dermatomyositis.

Cardiac involvement in dermatomyositis has been well described; myocarditis and cardiac arrhythmias are the most frequent manifestations. An 8-year-old girl is presented, who developed pericardial tamponade in the course of the disease. It is the first time this association has been reported.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2005

Immune responses to DNA mismatch repair enzymes hMSH2 and hPMS1 in patients with pancreatic cancer, dermatomyositis and polymyositis.

To identify tumor antigens useful for diagnosis and immunotherapy of patients with pancreatic ductal adenocarcinoma, we applied a SEREX approach with a cDNA library made from 5 pancreatic cancer cell lines and sera obtained from 8 patients with pancreatic cancer, and isolated total 32 genes, including 14 previously characterized genes and 18 genes with unknown functions. Among these isolated antigens, serum IgG antibodies for 2 isolated DNA mismatch repair enzymes, Homo sapiens mutS homolog 2 (hMSH2) and Homo sapiens postmeiotic segregation increased 1 (hPMS1), were detected in patients with pancreatic ductal adenocarcinoma and dermatomyositis (DM), and polymyositis (PM), but not in sera from healthy individuals. Immunohistochemical study demonstrated that hMSH2 and hPMS1 were over-expressed in pancreatic ductal adenocarcinoma compared to normal pancreatic ducts. These results suggested that hMSH2 and hPMS1 may be useful as CD4+ helper T cell antigens for immunotherapy of pancreatic cancer patients and that serum IgG antibodies may be useful for diagnosis of patients with pancreatic ductal adenocarcinoma and DM/PM.

Adenocarcinoma↗
PubMed1983

Dermatomyositis and multiple malignancies in a patient treated with methotrexate.

Dermatomyositis (DM) has been linked to internal malignancy but this relationship is quite variable in respect to tumor type and time sequence. The occurrence of multiple cancers in an individual with DM is rare. Immunosuppressive agents, particularly methotrexate have been used increasingly in corticosteroid resistant cases or for their corticosteroid sparing effects. These agents have not been implicated in the development of malignancy in patients with DM. A patient with a uterine adenocarcinoma developed DM that despite tumor removal was not responsive to corticosteroids. She was successfully treated with methotrexate for 18 months. She has subsequently developed an adenocarcinoma of the breast. The relationship of malignancy and DM as well as the role of methotrexate in the development of the second primary will be discussed.

Adenocarcinoma↗
PubMed1986

Dermatomyositis and female malignancy.

Six female patients, all with dermatomyositis (DM) and malignancy, have been evaluated and followed over a period of up to 6 years. Five patients had malignancy prior to the diagnosis of DM. Two of these five patients had uterine endometrial adenocarcinoma and another two patients had cervical carcinomas (one each adenocarcinoma and squamous cell carcinoma), and one had adenocarcinoma of the breast. In these five patients there was no evidence of malignancy at the time of diagnosis of DM. One of these five patients had an additional primary malignancy (adenocarcinoma of the breast) which complicated her course. An ovarian adenocarcinoma was present in the sixth patient. The ovarian carcinoma was discovered because of progressive cutaneous disease, and resection and chemotherapy led to a partial control of the DM. The patient with adenocarcinoma of the cervix developed metastases which were discovered when the DM became resistant to therapy. Although DM can parallel the course of a malignant disease, this was not generally the case in this group of patients.

Adenocarcinoma↗
PubMed2002

Concomitant dermatomyositis and myasthenia gravis presenting with respiratory insufficiency.

We report a 28-year-old woman with a history of chronic immune-mediated hepatitis, in whom the simultaneous manifestation of dermatomyositis and myasthenia gravis resulted in severe neck extensor weakness and subacute respiratory insufficiency, followed by proximal muscle weakness and external ophthalmoplegia. Radiological signs of a thymoma were absent. The distinguishing clinical, electrophysiological, and biopsy findings are discussed. We suggest that an underlying immunoregulatory disorder was present, explaining the occurrence of three rare immune-mediated diseases in one patient.

Adult↗
PubMedDate not supplied

Spontaneous electrical muscle fiber activity in polymyositis and dermatomyositis.

Forty patients with polymyositis or dermatomyositis underwent detailed electromyographic evaluation. The paraspinal muscles of all patients were examined, as were several extremity muscles. The distribution of fibrillation potentials (FPs) in different muscles is discussed. FPs were most frequent in paraspinal muscles. We conclude that, for any patient suspected of having a myopathy, electromyographic examination should include the paraspinal muscles.

Action Potentials↗
PubMed1999

Association among somatic HPRT mutant frequency, peripheral blood T-lymphocyte clonality, and serologic parameters of disease activity in children with juvenile onset dermatomyositis.

Somatic mutant frequencies (Mf) were determined using the HPRT T-cell cloning assay of peripheral blood T-lymphocytes from 14 children with juvenile onset dermatomyositis (JDM). Serologic parameters, specifically muscle enzyme determinations in JDM subjects, were correlated with residual lnMf (delta) in these patients to compare T-cell activation with clinical parameters associated with JDM. In addition TCR analysis was performed to determine T-cell proliferation and clonality on 12 HPRT mutant isolates from two individuals with JDM. Statistically significant correlations were found between residual lnMf and the following serologic parameters: aldolase (r = 0.771, P = 0.015); CPK (r = 0.602, P = 0.023); and SGOT (r = 0.656, P = 0.011) in children with JDM. In addition, identical TCR gene rearrangements were identified in 86 and 40% of the HPRT mutant isolates from the two patient samples analyzed, which is a significantly higher level of clonality than the 10-15% expected in normal individuals. These data suggest that determining HPRT Mf can be a useful antigen-independent method of selecting clonally expanding T-lymphocytes in autoimmune disease where relevant antigens are unknown. Future analysis of HPRT mutant isolates from children with active myositis may increase our understand of the activated T-cells involved in this disease.

Adolescent↗
PubMed2001

Expression of TNFalpha by muscle fibers in biopsies from children with untreated juvenile dermatomyositis: association with the TNFalpha-308A allele.

Juvenile dermatomyositis (JDM) is the most common pediatric inflammatory myopathy. In patients with JDM, the A --> G polymorphism in the tumor necrosis factor alpha (TNFalpha)-308 promoter region (TNFalpha-308A) is associated with prolonged disease course and increased production of TNFalpha by peripheral blood mononuclear cells (Arthritis Rheum. 43, 2368-2377, 2000). Magnetic resonance imaging directed biopsies from 21 white children with untreated JDM were evaluated for TNFalpha expression. Using monoclonal antibody to TNFalpha, fresh frozen sections were processed by the standard immunohistochemical technique. We investigated the association among the expression of TNFalpha by muscle fibers, disease activity, duration of untreated disease, and the TNFalpha-308 polymorphism. Untreated children with JDM who had the TNFalpha-308A allele had an increased number of TNFalpha stained muscle fibers than children with the TNFalpha-308G allele (P = 0.001). There was no association with disease activity or duration of untreated disease. We speculate that muscle fiber production of TNFalpha provides a microenvironment in which TNFalpha acts synergistically with other mediators to prolong muscle fiber damage.

Alleles↗
PubMed2002

Increased plasma thrombospondin-1 (TSP-1) levels are associated with the TNF alpha-308A allele in children with juvenile dermatomyositis.

Vascular occlusion is more frequent in children with juvenile dermatomyositis (JDM) who have the TNF alpha-308A allele. One of the potent anti-angiogenic factors is thrombospondin-1 (TSP-1). This study investigated the association of the TNF alpha-308A allele with circulating levels of angiogenic mediators, TSP-1, and platelet factor 4 (PF4) using fresh, platelet-poor plasma (PPP). The TNF alpha-308A allele was characterized by PCR amplification and NcoI digestion. Concentrations of TSP-1 and PF4 in PPP from 31 JDM patients and 25 matched pediatric controls were determined by ELISA. The majority of the JDM children with the TNF alpha-308A allele (7/12) produced more TSP-1 than their TNF alpha-308G counterparts (P < 0.05), and their TSP-1 values were inversely related to those for PF4 (P < 0.0006). We conclude that the increased circulating concentrations of TSP-1 associated with the TNF alpha-308A allele suggest that this anti-angiogenic regulator may play a significant role in the augmented vascular occlusion observed in JDM children with this genetic marker.

Alleles↗
PubMed1995

Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark.

Polymyositis and dermatomyositis (PM/DM) have been associated with cancer, although the long-term risks are poorly understood. To evaluate the risk of cancer by time periods subsequent to PM/DM diagnosis, a cohort of 539 patients hospitalized with PM/DM in Denmark between 1977 and 1989 was identified from the Danish Central Hospital Discharge Register. Cancer incidence among cohort members was ascertained by linkage to the Danish Cancer Registry using a unique personal-identification number. The overall cancer risk was elevated significantly among patients with DM (standardized incidence ratio [SIR] = 3.8, 95 percent confidence interval [CI] = 2.6-5.4) and to a lesser extent PM (SIR = 1.7, CI = 1.1-2.4). Significant excesses were observed for cancers of lung, ovary, and lymphatic and hematopoietic system. However, the excess cancer incidence declined steadily with increasing years since initial diagnosis of PM/DM. The cancer risk was increased about sixfold (SIR = 5.9, CI = 3.8-8.7) during the first year, but was lower during the second year (SIR = 2.5, CI = 1.1-4.8), with no significant excesses in subsequent years of follow-up. These findings confirm that PM/DM may occur as a paraneoplastic syndrome that calls for steps aimed at early cancer detection and treatment. Among long-term survivors of PM/DM, however, there is little evidence to warrant extensive preventive and screening measures beyond those recommended for the general population.

Adult↗
PubMed1994

Serial magnetic resonance imaging in juvenile dermatomyositis--delayed normalization.

Demonstration of myositis in juvenile dermatomyositis (JDM) has traditionally required the presence of two out of three objective findings including muscle enzyme studies, electromyography, and muscle biopsy. Magnetic resonance imaging (MRI) is considered another objective criterion and has been recommended for follow-up studies as well. We report on a 6-year-old girl with JDM, presenting with facial rash and proximal muscle weakness, in whom MRI, in conjunction with elevated creatinine kinase (CK) levels, was diagnostic, with an increased T2 signal of proximal muscles. MRI reflected exacerbation of myositis in spite of steroid therapy 2 weeks later, but failed to mirror normalization of muscle strength and CK values 10 weeks later. In fact, improvement of MRI followed clinical and laboratory normalization with a delay of 2 months. This possible delay should be considered when MRI is used to monitor the response to therapy.

Child↗
PubMed1989

The heart in polymyositis-dermatomyositis.

Fourteen patients with polymyositis-dermatomyositis (PM-DM) underwent prospective cardiac assessment with non-invasive techniques. One patient had electrocardiographic evidence of Long-Ganong-Levine syndrome with multiple supraventricular premature beats. Echocardiographically one patient had late systolic prolapse of the posterior mitral leaflet and another had paradoxical movements of the interventricular septum. The study suggests that cardiac involvement is infrequent in PM-DM.

Adult↗
PubMed1979

An electron microscopic study of childhood dermatomyositis.

Muscle biopsies from 12 patients with a typical clinical picture of dermatomyositis have been examined by electron microscopy. Endothelial cells of intramuscular blood vessels, their basal lamina, pericytes, muscle fibers, and satellite cells show degenerative or regenerative alterations. In nine patients, tubular arrays were noted in the cisterns of endoplasmic reticulum of endothelial cells, pericytes, lymphocytes, macrophages and satellite cells. Other types of inclusions were also observed. The pathogenesis of the disease is discussed.

Adolescent↗
PubMed1982

Dermatomyositis as an immunologic complication of toxoplasmosis.

We present immunohistochemical, light- and electron-microscopic findings on a muscle biopsy specimen from a 21-year-old woman who developed debilitating dermatomyosisits in the course of toxoplasmosis. The muscle showed perifascicular muscle cell atrophy and prominent ultrastructural changes consistent with polymyositis. These myopathic changes were interpreted as an immunologic complication of systemic toxoplasmosis and were related to immunohistochemically demonstrable immune complex deposits in the small blood vessels. Our data suggest that dermatomyositis is caused and/or related to Toxoplasma infection and is an immune complex-mediated systemic disease.

Adult↗