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At least 631 records · Page 35Linked to original sources
PubMed2003

Dermatomyositis and myelodysplastic syndrome with myelofibrosis responding to methotrexate therapy.

Dermatomyositis (DM) has not yet been reported as a complication of myelodysplastic syndrome (MDS). A 50-year-old man was diagnosed as having MDS because of the presence of anemia, the appearance of immature cells in peripheral blood, and the abnormal cellular morphology. A few months later, high fever, myalgia and erythema developed. Although DM symptoms were resistant to high-dose corticosteroid administration, methotrexate (MTX) therapy improved not only the symptoms of DM but also hematologic findings related to MDS. This indicates that immunosuppressive therapy including MTX administration can be useful for patients with MDS with autoimmune symptoms.

Blood Cells↗
PubMed1989

Evidence for a novel picornavirus in human dermatomyositis.

We prepared RNA probes from cloned segments of human and murine enteroviruses (EVs) for in situ hybridization of skeletal muscle biopsies from patients with dermatomyositis (DM), polymyositis, other inflammatory myopathies, and noninflammatory muscle diseases, and from normal control subjects. A probe derived from Theiler's murine encephalomyelitis virus (TMEV) detected viral RNA within mononuclear cells of the interstitial connective tissue in 3 of 5 patients with adult-onset DM. None of these patients showed positive hybridization to probes derived from human EVs (poliovirus type 1 and Coxsackie virus B3) applied to subjacent sections of the same biopsies. The remaining 2 adult DM patients, 4 patients with childhood-onset DM, and 24 non-DM patients did not react with either TMEV or human enterovirus probes. Histochemical stains for esterase and immunoperoxidase stains for Mac-1 antigen in the 3 DM patients who reacted positively revealed positive cells in the same distribution as, but in far greater number than, those positive by in situ hybridization. Immunoperoxidase staining for HLA-DR antigens revealed positive cells in the same distribution and number as were seen with the TMEV probe. We conclude that an EV-like agent, more closely related to TMEV than to human EVs, may be associated with DM and that this agent is probably localized within muscle macrophages that express class II major histocompatibility complex antigens.

Adult↗
PubMed2002

Interleukin-1alpha expression in capillaries and major histocompatibility complex class I expression in type II muscle fibers from polymyositis and dermatomyositis patients: important pathogenic features independent of inflammatory cell clusters in muscle tissue.

OBJECTIVE: To address the hypothesis that endothelial cells and/or muscle fibers are primary targets in the disease process by analysis of muscle tissue from patients with polymyositis (PM) and dermatomyositis (DM). METHODS: We included patients with laboratory signs and clinical symptoms typical of myositis, but without detectable infiltration of clusters of inflammatory cells in their muscle biopsy samples. An immunohistochemical technique was applied to identify CD3, CD68, lymphocyte function-associated antigen 1alpha, CD11b, very late activation antigen 4, endothelium 4, interleukin-1alpha (IL-1alpha), intercellular adhesion molecule 1, vascular cell adhesion molecule 1, IgG, IgM, IgA, and HLA-A/B/C in muscle tissue. Fiber type was defined by ATPase staining. RESULTS: IL-1alpha expression was detected in endothelial cells of capillaries to a greater extent in patients than in controls, and class I major histocompatibility complex (MHC) expression was significantly increased in muscle fibers. We also observed that class I MHC expression was mainly confined to type II muscle fibers. CONCLUSION: Our findings imply that defined molecular changes of blood vessels and muscle fibers are both independent of adjacent inflammatory infiltrates and could thus be primary events in the development of myositis. Moreover, both IL-1alpha and class I MHC molecules might be important for the development of clinical symptoms in PM and DM patients.

Adult↗
PubMed2002

Decreased aerobic capacity in children with juvenile dermatomyositis.

OBJECTIVE: To determine whether patients with juvenile dermatomyositis (DM) have limited aerobic capacity compared with healthy controls. METHODS: Fourteen juvenile DM patients with inactive to moderately active, stable disease (age range 7-17 years) and 14 age- and sex-matched controls performed a maximal exercise test using a cycle ergometer. Oxygen uptake and power were measured at peak exercise (VO(2peak) and W(peak), respectively) and at anaerobic threshold (AT and W(AT)). Juvenile DM disease activity and damage were also assessed. RESULTS: Patients with juvenile DM had significantly reduced VO(2peak) (19.6 ml O(2)/kg/minute in juvenile DM versus 31.1 ml O(2)/kg/minute in controls), peak heart rate (166 versus 184 beats per minute), W(peak) (1.6 versus 2.7 watts/kg), AT (11.1 versus 18.0 ml O(2)/kg/minute) and W(AT) (0.6 versus 1.4 watts/kg), compared to controls (P <or= 0.05 for each). Aerobic exercise parameters correlated with physician global disease activity and damage, T1-weighted magnetic resonance imaging, and Childhood Myositis Assessment Scale scores (r(s) = 0.58 - 0.82, P <or= 0.05). CONCLUSION: Patients with juvenile DM with a range of disease activity have a decreased aerobic and work capacity compared to healthy children. Aerobic exercise limitation in juvenile DM correlates best with measures of disease damage (global damage assessment, T1-weighted magnetic resonance imaging, and disease duration). Aerobic exercise testing may be valuable in the assessment of physical endurance, and aerobic training may be indicated as part of the therapeutic regimen in myositis patients with inactive to moderately active, stable disease.

Adolescent↗
PubMed2002

Self epitopes shared between human skeletal myosin and Streptococcus pyogenes M5 protein are targets of immune responses in active juvenile dermatomyositis.

OBJECTIVE: To identify self T cell epitopes associated with proinflammatory immune responses and clinically active juvenile dermatomyositis (juvenile DM). The target of our search for relevant epitopes was represented by amino acid sequences shared between human skeletal myosin and Streptococcus pyogenes M5 protein. The long-term objective of the project is to identify suitable targets for immunotherapy of the disease. METHODS: We used computerized algorithms to identify putative agretopes on both the human myosin and Streptococcus M5 proteins. Direct binding assays for homolog peptides were used to confirm such predictions. Antigenicity and functional cross-reactivity were evaluated by cytotoxicity assays and by measurement of cytokine levels. Specific T cells were isolated by T cell capture, and T cell receptor (TCR) V(beta) gene usage was identified by reverse transcriptase-polymerase chain reaction. RESULTS: We identified peptides that are targets of disease-specific cytotoxic T cell responses. T cell reactivity against the self peptides correlates with clinical signs of early, active myositis. Such reactivity is accompanied by production of proinflammatory cytokines, which may contribute to the damage. T cell cross-recognition of bacterial and human homologs was shown functionally as well as by sorting peptide-specific T cells and identifying oligoclonal and largely overlapping TCR V(beta) gene usage. CONCLUSION: These findings represent the first identification of a self epitope in juvenile DM, providing a potential candidate for antigen-specific immune therapy.

Bacterial Proteins↗
PubMed2002

Interstitial lung disease in polymyositis and dermatomyositis.

OBJECTIVES: To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM. METHODS: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. RESULTS: Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent. CONCLUSION: Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.

Antibodies, Antinuclear↗
PubMed2003

Disease activity score for children with juvenile dermatomyositis: reliability and validity evidence.

OBJECTIVE: To assess the reliability and validity of the Disease Activity Score (DAS), an instrument used to evaluate children with juvenile dermatomyositis (JDM). METHODS: Psychometric study of internal consistency, reliability, rater agreement, and the relationship with measures of muscle strength and disability was conducted. RESULTS: The DAS ratings are internally consistent (reliability = 0.89) and describe a wide range of disease activity. The pediatric rheumatologists in this study agree on the presence of most of the disease indicators. Their disagreements tend to cancel each other, resulting in highly correlated (r = 0.79) overall measures across raters. Estimates of muscle weakness using the DAS and ratings of muscle strength obtained independently from therapists are highly related (r = -0.77), but estimates of disease activity and disability are weakly related (r = 0.20). CONCLUSION: The DAS exhibits evidence of good reliability and validity. The combination of skin and muscle strength assessments makes this easily administered instrument a useful addition in the evaluation of children with JDM.

Adolescent↗
PubMed2003

Bone mineral density in juvenile dermatomyositis: assessment using dual x-ray absorptiometry.

OBJECTIVE: To determine the bone mineral density (BMD) status of our juvenile dermatomyositis (DM) population and to compare the frequency of osteopenia in patients with active disease requiring corticosteroids with that in patients with inactive disease who are not receiving corticosteroids. METHODS: Medical charts of all children diagnosed as having juvenile DM at our institution between 1989 and 1999 were reviewed for demographic and clinical data, including disease activity and duration of corticosteroid therapy. BMD measurements of the lumbar spine (L1-L4) were performed using dual x-ray absorptiometry (DXA). Z scores were calculated from the BMD data for comparison with published normative data. RESULTS: A total of 15 patients were assessed: 10 with active disease, and 5 with inactive disease who had not taken corticosteroids for an average of 6.0 years (range 3.4-8.1 years). Baseline BMD measurements demonstrated osteopenia or frank osteoporosis in the majority of patients, including 6 of the 10 patients with active disease and 4 of the 5 patients whose disease was in remission. Fourteen patients had serial BMD measurements. Persistent or worsening osteopenia was documented in all patients who had ongoing active disease, except for 3 patients who had been treated with bisphosphonates because of vertebral compression fractures. CONCLUSION: Osteopenia is common in patients with juvenile DM, and it usually worsens with ongoing disease. It can persist for many years after the disease enters remission. Bisphosphonates appeared to beneficially affect bone mineralization in our patients. Treatment to prevent the long-term complications of osteoporosis in patients with juvenile DM should be considered and requires further study.

Absorptiometry, Photon↗
PubMed2004

Anatomic localization of immature and mature dendritic cell subsets in dermatomyositis and polymyositis: Interaction with chemokines and Th1 cytokine-producing cells.

OBJECTIVE: To clarify the involvement of dendritic cells (DCs), chemokines, and proinflammatory Th1 cytokines in the pathogenesis of the chronic muscle diseases dermatomyositis (DM) and polymyositis (PM). METHODS: We characterized by immunohistochemistry the DC subsets and their interaction with cells producing chemokines and the Th1 cytokines interleukin-17 (IL-17) and interferon-gamma (IFNgamma). Immature and mature DCs were defined by the expression of CD1a and DC-LAMP/CD83, respectively. RESULTS: Immature DCs were mainly detected in lymphocytic infiltrates in DM and PM muscle tissue samples. Mature DCs were detected in perivascular infiltrates and surrounded muscle fibers. IL-17-positive and IFNgamma-positive cells were also observed in perivascular infiltrates in both cases. We then focused on the expression of the CCL20/CCR6 chemokine/receptor complex, which controls immature DC migration, and on the expression of the CCL19/CCR7 and CCL21/CCR7 chemokine/receptor complexes, which control mature DC migration. CCL20 and CCR6 colocalized in lymphocytic infiltrates in DM and PM samples. CCL21 was rarely observed in DM samples and never observed in PM samples. CCL19- and CCR7-expressing cells were absent in both tissues. CONCLUSION: The close association between CCL20/CCR6 and immature DCs suggests the contribution of CCL20 to CCR6+ immature DC homing. Detection of mature DCs in DM and PM muscle tissue samples despite the lack of CCL19 and CCR7 is evidence for a local maturation of DCs in inflammatory muscle tissue without lymphoid organ organization.

Adult↗
PubMed1980

Heterogeneity of precipitating antibodies in polymyositis and dermatomyositis. Characterization of the Jo-1 antibody system.

Precipitating antibodies occurring in 26 "pure" polymyositis (PM) patients and 22 "pure" dermatomyositis (DM) patients were studied by double immunodiffusion using calf thymus nuclear extract and whole calf thymus extract. Seventeen of 26 (65%) PM sera and 13 of 22 (59%) DM sera showed positive reactions, and 18, 10, and 2 sera exhibited 1, 2, and 3 precipitin lines, respectively. Common specificities were established by immunodiffusion for the 11 PM and 6 DM sera with strong precipitin lines. These were classed into three groups of shared specificity and six reactions specific for individual sera. These 9 calf thymus antigens were shown to be different from the known soluble tissue antigens Sm, nuclear RNP, Ro, and La. A 600-fold purification of the most frequently appearing system designated Jo-1 was achieved and its clinical specificity for patients with PM syndromes was established.

Antibodies↗
PubMed1983

Mortality from polymyositis and dermatomyositis in the United States, 1968-1978.

Mortality from polymyositis and dermatomyositis was estimated between 1968 and 1978. Age-specific average annual mortality rates showed unimodal distributions for all sex-race groups. Synergistic interaction was demonstrated between female sex and nonwhite race, greatest mortality being in nonwhite females through age 74. Increases in annual death rates occurred among both white males and white females during the interval. Finally, the increase in mean age at death among all sex groups correlated with improved prognosis as well as decreased mortality among younger persons over time.

Adolescent↗
PubMed1985

Immunogenetic studies of juvenile dermatomyositis. III. Study of antibody to organ-specific and nuclear antigens.

Ninety children with definite juvenile dermatomyositis (JDMS), who had been HLA typed, were tested for the presence of tissue or organ-specific antibodies. Sixty had active disease at the time of study. The mean disease duration was 4 years, and 30 had soft tissue calcifications. The following autoantibodies were sought: thyroid, gastric parietal cells, smooth muscle, striated muscle, microsomes, mitochondria, DNA, extractable nuclear antigen, Sm, PM-1, antinuclear antibody (ANA), and rheumatoid factor. Only the ANA and PM-1 were more frequent in patients than in controls (P less than 0.0002 and P less than 0.001, respectively). Higher levels of immune complexes (P less than 0.01) were found in sera from patients with JDMS than in sera from controls and were correlated with the presence of ANA in patients (P less than 0.01). Soft tissue calcification was not associated with any autoantibody or HLA antigen, but with disease duration and activity (P less than 0.001 and P less than 0.05, respectively). There was no association between the occurrence of any autoantibody and the presence of HLA-B8 or DR3 among the white patients with JDMS. The frequency of autoantibodies in 43 full siblings of children with JDMS was not increased. We conclude that children with JDMS, with or without HLA-B8/DR3, do not show evidence of a generalized nonspecific antibody response to tissue antigens. The significance of the increased antibody to nuclear antigens ANA and PM-1 remains to be determined.

Adolescent↗
PubMed1985

The association between Mi-2 antibodies and dermatomyositis.

Antibodies to Mi, an antigen in calf thymus extract, have been demonstrated by complement fixation inhibition in polymyositis (PM) and dermatomyositis (DM) sera but not in the sera of individuals without myositis. The original Mi reference serum defined 2 precipitating antibodies, using immunodiffusion (ID). Anti-Mi-1 was not active in complement fixation. We have now studied in further detail anti-Mi-2, which appears to be the antibody in Mi serum that fixes complement. Mi-2 antigen was purified by immuno-affinity chromatography. An enzyme-linked immunosorbent assay (ELISA) to measure Mi-2 antibody, using this antigen, was used to test the sera of 139 myositis patients: 52 had DM and 87 had PM. Control sera from 35 normal subjects and 93 patients with other connective tissue diseases were also tested. Only 13 sera were considered definitely positive for anti-Mi-2. All were from patients who had myositis, 11 of whom had DM. Only DM sera had anti-Mi-2 by ID, and all sera with anti-Mi-2 by ID were positive by ELISA. A number of other sera, including many from patients with other connective tissue diseases and 2 from normal subjects (all without precipitating antibodies) had lower elevations which were of uncertain significance. Detection of anti-Mi-2 by ID as well as by ELISA was significantly more frequent in DM than in PM. Anti-Mi-2 appears to be closely linked to DM, and is the first specific serologic marker for this form of myositis.

Antibody Specificity↗
PubMed1986

Effect of warfarin sodium therapy on excretion of 4-carboxy-L-glutamic acid in scleroderma, dermatomyositis, and myositis ossificans progressiva.

The effect of warfarin sodium on excretion of calcium, phosphorus, and 4-carboxy-L-glutamic acid (Gla) was studied in 5 patients with ectopic calcification (2 with scleroderma, 1 with dermatomyositis, and 2 with myositis ossificans progressiva). Warfarin reduced urinary excretion of Gla in all patients, but no changes in calcium and phosphorus excretion or in objective parameters of calcinosis were observed during 6-36 months of treatment. Two patients experienced hemorrhagic complications during therapy, emphasizing a hazard of long-term anticoagulation treatment. Since ectopic calcium deposits contain Gla-rich protein, suppression of Gla synthesis by warfarin sodium over a longer period may prevent deposition and allow removal of existing calcinosis deposits.

Adult↗
PubMed1987

Intravenous pulse methylprednisolone treatment of juvenile dermatomyositis.

Recent observations that juvenile dermatomyositis (JDMS) may be a heterogenous disorder, in both its clinical course and prognosis, suggest that subgroups of patients with this disease may respond to various modes of therapy. Therefore, current treatment recommendations of daily, long-term oral corticosteroid treatment, with its attendant side effects, may not be necessary for all children with JDMS. We report the results of treatment with high-dose, pulse intravenous methylprednisolone in 7 children with JDMS, 3 of whom had an excellent response with complete remission and no need for daily steroid therapy. We suggest that patients with early, mild JDMS may be candidates for therapy with intravenous methylprednisolone.

Adolescent↗
PubMed1987

Activation of complement and coagulation in juvenile dermatomyositis.

In patients with juvenile dermatomyositis (JDM), small vessel occlusion and thrombosis result in a decrease in the capillary: muscle fiber ratio. We studied 15 patients with JDM. Six of 7 patients with clinically active JDM had elevated levels of C3d. Moreover, concentrations of fibrinopeptide A and factor VIII-related antigen were significantly increased in patients with clinically active JDM.

Adolescent↗
PubMed1988

Nailfold capillary microscopy in polymyositis and dermatomyositis.

Nailfold capillary microscopy was performed in 19 patients with polymyositis (PM) and 16 with dermatomyositis (DM). Enlarged capillary loops were seen more frequently in patients with DM (56%) than in those with PM (21%). Avascular lesions occurred in both PM and DM, but were more prevalent and more severe in patients with DM. Raynaud's phenomenon, arthritis, and pulmonary involvement were associated with greater numbers of enlarged capillary loops and more severe avascular lesions. The severity of the observed microvascular abnormalities did not correlate with the occurrence of malignancy or active myositis, but appeared to lessen with prolonged disease remission.

Capillaries↗
PubMed1991

Serial magnetic resonance imaging in juvenile dermatomyositis.

Magnetic resonance imaging (MRI) was used to follow the course of juvenile dermatomyositis from the onset of disease through resolution of a primary relapse. The signal intensity of the T2-weighted image of involved muscles was elevated during periods of disease activity, and returned to approximately normal levels with effective suppression of disease activity. T1-weighted images of involved muscles remained approximately normal despite disease activity.

Child, Preschool↗