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Arthritis & Rheumatology (Hoboken, N.J.) May 2017To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM).
2016 American College of Rheumatology/European League Against Rheumatism Criteria for Minimal, Moderate, and Major Clinical Response in Adult Dermatomyositis and Polymyositis: An International Myositis Assessment and Clinical Studies Group/Paediatric Rheumatology International Trials Organisation...
OBJECTIVE
To develop response criteria for adult dermatomyositis (DM) and polymyositis (PM).
METHODS
Expert surveys, logistic regression, and conjoint analysis were used to develop 287 definitions using core set measures. Myositis experts rated greater improvement among multiple pairwise scenarios in conjoint analysis surveys, where different levels of improvement in 2 core set measures were presented. The PAPRIKA (Potentially All Pairwise Rankings of All Possible Alternatives) method determined the relative weights of core set measures and conjoint analysis definitions. The performance characteristics of the definitions were evaluated on patient profiles using expert consensus (gold standard) and were validated using data from a clinical trial. The nominal group technique was used to reach consensus.
RESULTS
Consensus was reached for a conjoint analysis-based continuous model using absolute percent change in core set measures (physician, patient, and extramuscular global activity, muscle strength, Health Assessment Questionnaire, and muscle enzyme levels). A total improvement score (range 0-100), determined by summing scores for each core set measure, was based on improvement in and relative weight of each core set measure. Thresholds for minimal, moderate, and major improvement were ≥20, ≥40, and ≥60 points in the total improvement score. The same criteria were chosen for juvenile DM, with different improvement thresholds. Sensitivity and specificity in DM/PM patient cohorts were 85% and 92%, 90% and 96%, and 92% and 98% for minimal, moderate, and major improvement, respectively. Definitions were validated in the clinical trial analysis for differentiating the physician rating of improvement (P < 0.001).
CONCLUSION
The response criteria for adult DM/PM consisted of the conjoint analysis model based on absolute percent change in 6 core set measures, with thresholds for minimal, moderate, and major improvement.
Topics: Alanine Transaminase; Antirheumatic Agents; Aspartate Aminotransferases; Creatine Kinase; Dermatomyositis; Europe; Fructose-Bisphosphate Aldolase; Humans; L-Lactate Dehydrogenase; Logistic Models; Muscle Strength; Outcome Assessment, Health Care; Patient Reported Outcome Measures; Polymyositis; Rheumatology; Societies, Medical; Surveys and Questionnaires; Treatment Outcome; United States
PubMed: 28382787
DOI: 10.1002/art.40064 -
Internal Medicine (Tokyo, Japan) Aug 2022
Topics: Dermatomyositis; Foot; Humans; Skin
PubMed: 35110493
DOI: 10.2169/internalmedicine.8999-21 -
International Journal of Environmental... Apr 2021Polymyositis (PM) and dermatomyositis (DM) are autoimmune-mediated multisystemic myopathies, characterized mainly by proximal muscle weakness. A connection between...
Polymyositis (PM) and dermatomyositis (DM) are autoimmune-mediated multisystemic myopathies, characterized mainly by proximal muscle weakness. A connection between epilepsy and PM/DM has not been reported previously. Our study aim is to evaluate this association. A case-control study was conducted, enrolling a total of 12,278 patients with 2085 cases (17.0%) and 10,193 subjects in the control group (83.0%). Student's t-test was used to evaluate continuous variables, while the chi-square test was applied for the distribution of categorical variables. Log-rank test, Kaplan-Meier curves and multivariate Cox proportional hazards method were performed for the analysis regarding survival. Of the studied 2085 cases, 1475 subjects (70.7%) were diagnosed with DM, and 610 patients (29.3%) with PM. Participants enrolled as cases had a significantly higher rate of epilepsy ( = 48 [2.3%]) as compared to controls ( = 141 [1.4%], < 0.0005). Using multivariable logistic regression analysis, PM was found only to be significantly associated with epilepsy (OR 2.2 [95%CI 1.36 to 3.55], = 0.0014), whereas a non-significant positive trend was noted in DM (OR 1.51 [95%CI 0.99 to 2.30], = 0.0547). Our data suggest that PM is associated with a higher rate of epilepsy compared to controls. Physicians should be aware of this comorbidity in patients with immune-mediated myopathies.
Topics: Case-Control Studies; Comorbidity; Cross-Sectional Studies; Dermatomyositis; Epilepsy; Humans; Polymyositis
PubMed: 33920065
DOI: 10.3390/ijerph18083983 -
European Journal of Medical Research Feb 2019This study aims to detect serum levels of monocyte chemoattractant protein-1 (MPC-1) and transforming growth factor-β1 (TGF-β1) in polymyositis/dermatomyositis (PM/DM)...
OBJECTIVE
This study aims to detect serum levels of monocyte chemoattractant protein-1 (MPC-1) and transforming growth factor-β1 (TGF-β1) in polymyositis/dermatomyositis (PM/DM) patients complicated with interstitial lung disease (ILD), to reveal the significance of the changes in these levels in the pathogenesis of PM/DM complicated with ILD.
METHODS
Serum MCP-1 and TGF-β1 levels in PM/DM patients complicated with ILD, patients with pulmonary infections and normal controls (n = 30, each) were detected using enzyme-linked immunosorbent assay (ELISA), and the correlation between PM/DM complicated with ILD and serum MCP-1 and TGF-β1 levels was analyzed.
RESULTS
Serum MCP-1 and TGF-β1 levels were both higher in PM/DM patients complicated with ILD compared with patients with pulmonary infections and normal controls.
CONCLUSION
Serum MCP-1 and TGF-β1 levels increased in PM/DM patients, and were closely correlated to the complication of ILD. This finding can be used for distinguishing between pulmonary infections and ILD, providing a new diagnostic method for the early prediction of DM/PM complicated with ILD.
Topics: Adult; Chemokine CCL2; Dermatomyositis; Female; Humans; Male; Polymyositis; Transforming Growth Factor beta1
PubMed: 30764873
DOI: 10.1186/s40001-019-0368-7 -
Drug Research Nov 2015
Topics: Dermatomyositis; Humans; Myositis; Myositis, Inclusion Body; Polymyositis
PubMed: 26536184
DOI: 10.1055/s-0035-1558068 -
The Journal of Rheumatology Jan 2021Patients with dermatomyositis (DM) and polymyositis (PM) have reduced muscle endurance.The aim of this study was to streamline the Functional Index-2 (FI-2) by...
OBJECTIVE
Patients with dermatomyositis (DM) and polymyositis (PM) have reduced muscle endurance.The aim of this study was to streamline the Functional Index-2 (FI-2) by developing the Functional Index-3 (FI-3) and to evaluate its measurement properties, content and construct validity, and intra- and interrater reliability.
METHODS
A dataset of the previously performed and validated FI-2 (n = 63) was analyzed for internal redundancy, floor, and ceiling effects. The content of the FI-2 was revised into the FI-3. Construct validity and intrarater reliability of FI-3 were tested on 43 DM and PM patients at 2 rheumatology centers. Interrater reliability was tested in 25 patients. The construct validity was compared with the Myositis Activities Profile (MAP), Health Assessment Questionnaire (HAQ), and Borg CR-10 using Spearman correlation coefficient.
RESULTS
Spearman correlation coefficients of 63 patients performing FI-3 revealed moderate to high correlations between shoulder flexion and hip flexion tasks and similar correlations with MAP and HAQ scores; there were lower correlations for neck flexion task. All FI-3 tasks had very low to moderate correlations with the Borg scale. Intraclass correlation coefficients (ICC) of FI-3 tasks for intrarater reliability (n = 25) were moderate to good (0.88-0.98). ICC of FI-3 tasks for interrater reliability (n = 17) were fair to good (range 0.83-0.96).
CONCLUSION
The FI-3 is an efficient and valid method for clinically assessing muscle endurance in DM and PM patients. FI-3 construct validity is supported by the significant correlations between functional tasks and the MAP, HAQ, and Borg CR-10 scores.
Topics: Dermatomyositis; Humans; Polymyositis; Range of Motion, Articular; Reproducibility of Results
PubMed: 32295854
DOI: 10.3899/jrheum.191374 -
Medicine Sep 2021The aim of this study was to evaluate the association between clinical phenotypes of dermatomyositis (DM) and polymyositis (PM) with myositis-specific antibodies (MSAs),...
The aim of this study was to evaluate the association between clinical phenotypes of dermatomyositis (DM) and polymyositis (PM) with myositis-specific antibodies (MSAs), and overlap diagnosis of systemic autoimmune diseases.This cross-sectional study was conducted on 67 patients with DM and 27 patients with PM recruited from a regional hospital in southern Taiwan. Clinical phenotypes of DM and PM were assessed and MSAs were measured using a commercial line blot assay. The association of clinical phenotypes of DM and PM with MSAs and overlap diagnosis of systemic autoimmune diseases was performed using univariate and multiple logistic regression analyses.Clinically, patients with DM and PM and overlap diagnosis of systemic sclerosis were associated with a higher risk of interstitial lung diseases (ILDs) (odds ratio [OR] = 6.73; P = .048), Raynaud phenomenon (OR = 7.30; P = .034), and malignancy (OR = 350.77; P = .013). The risk of malignancy was also associated with older age (OR 1.31; P = .012), and male patients were associated with a higher risk of fever. For MSAs, anti-aminoacyl-tRNA synthetase antibodies were associated with ILD, antinuclear antibody were associated with a lower risk of arthritis, anti-transcription intermediary factor 1-gamma antibodies were associated with milder symptoms of muscle weakness, anti-Ku antibodies were associated with overlap diagnosis of systemic lupus erythematosus, and anti-Ro52 antibodies were associated with the development of Raynaud phenomenon and Sjögren syndrome.MSAs and overlap diagnosis of systemic sclerosis were significantly associated with clinical phenotypes of DM and PM. Physicians should be vigilant for malignancy in older DM and PM patients with overlap diagnosis of systeic sclerosis. The possibility of developing ILD in patients with overlap diagnosis of systemic sclerosis or serum positivity of anti-aminoacyl-tRNA synthetase antibodies should be considered.
Topics: Adult; Aged; Autoantibodies; Biomarkers; Cross-Sectional Studies; Dermatomyositis; Female; Humans; Male; Middle Aged; Phenotype; Polymyositis; Taiwan
PubMed: 34664863
DOI: 10.1097/MD.0000000000027230 -
Frontiers in Immunology 2022Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5 DM) is an infrequent autoimmune disease, which mainly distributes in Asians and... (Review)
Review
Anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5 DM) is an infrequent autoimmune disease, which mainly distributes in Asians and females. MDA5 DM usually presents various skin lesions and positive anti-MDA5 antibody (a myositis-specific autoantibody for itself) with amyopathic or hypomyopathic features. For MDA5 DM patients, rapidly progressive interstitial lung disease is a common complication with a high-speed deterioration and a poor prognosis. Besides, there are other complications of MDA5 DM patients, including pneumomediastinum, macrophage activation syndrome and spontaneous intramuscular hemorrhage. These complications were rare but lethal, so it is necessary to explore their diagnosis methods, therapies and potential mechanisms, which are helpful for early diagnoses and timely treatment. To date, several cases and studies have shown distinctive features, diagnoses and treatments of these three rare complications, and there are also some differences among them. In this review, we outlined the characteristics, administration and potential pathogenesis of these rare complications of MDA5 DM.
Topics: Female; Humans; Interferon-Induced Helicase, IFIH1; Dermatomyositis; Autoantibodies; Myositis; Hemorrhage
PubMed: 36275649
DOI: 10.3389/fimmu.2022.1009546 -
Frontiers in Immunology 2022Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Most of the infected individuals...
Coronavirus disease 2019 (COVID-19) is an infectious disease caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Most of the infected individuals have recovered without complications, but a few patients develop multiple organ involvements. Previous reports suggest an association between COVID-19 and various inflammatory myopathies, in addition to autoimmune diseases. COVID-19 has been known to exacerbate preexisting autoimmune diseases and trigger various autoantibodies and autoimmune disease occurrence. Here we report a case of complicated COVID-19 with anti-synthetase autoantibodies (ASSs) presenting with skin rash, muscle weakness, and interstitial lung disease (ILD) and subsequently diagnosed with dermatomyositis (DM). A 47-year-old Japanese male patient without any previous history of illness, including autoimmune diseases, presented with a high fever, sore throat, and cough. Oropharyngeal swab for SARS-Cov-2 polymerase chain reaction tested positive. He was isolated at home and did not require hospitalization. However, his respiratory symptoms continued, and he was treated with prednisolone (20 mg/day) for 14 days due to the newly developing interstitial shadows over the lower lobes of both lungs. These pulmonary manifestations remitted within a week. He presented with face edema and myalgia 4 weeks later when he was off corticosteroids. Subsequently, he presented with face erythema, V-neck skin rash, low-grade fever, and exertional dyspnea. High-resolution computed tomography of the chest showed ILD. Biochemical analysis revealed creatine kinase and aldolase elevations, in addition to transaminases. Anti-aminoacyl tRNA synthetase (ARS) was detected using an enzyme-linked immunosorbent assay (170.9 U/mL) (MESACUP™ (Medical & Biological Laboratories, Japan), and the tRNA component was identified as anti-PL-7 and anti-Ro-52 antibodies using an immunoblot assay [EUROLINE Myositis Antigens Profile 3 (IgG), Euroimmun, Lübeck,Germany]. The patient was diagnosed with DM, especially anti- synthase antibody syndrome based on the presence of myositis-specific antibodies, clinical features, and pathological findings. The present case suggests that COVID-19 may have contributed to the production of anti-synthetase antibodies (ASAs) and the development of DM. Our case highlights the importance of the assessment of patients who present with inflammatory myopathy post-COVID-19 and appropriate diagnostic work-up, including ASAs, against the clinical features that mimic DM after post-COVID-19.
Topics: Humans; Male; Middle Aged; Dermatomyositis; COVID-19; SARS-CoV-2; Myositis; Lung Diseases, Interstitial; Autoantibodies; Autoimmune Diseases; Exanthema
PubMed: 36353621
DOI: 10.3389/fimmu.2022.1002329 -
Scientific Reports Feb 2021Our study aimed to investigate the incidence, risk factors and time to occurrence of malignancy in patients with dermatomyositis (DM) and polymyositis (PM). The...
Our study aimed to investigate the incidence, risk factors and time to occurrence of malignancy in patients with dermatomyositis (DM) and polymyositis (PM). The electronic medical records of 1100 patients with DM and 1164 patients with PM were studied between January 2001 and May 2019. Malignancies after myositis were diagnosed in 61 (5.55%) patients with DM and 38 (3.26%) patients with PM. The cumulative incidence of malignancies in patients with DM were significantly higher than patients with PM (hazard ratio = 1.78, log-rank p = 0.004). Patients with DM had a greater risk of developing malignancy than those with PM at 40-59 years old (p = 0.01). Most malignancies occurred within 1 year after the initial diagnosis of DM (n = 35; 57.38%). Nasopharyngeal cancer (NPC) was the most common type of malignancy in patients with DM (22.95%), followed by lung, and breast cancers. In patients with PM, colorectal, lung and hepatic malignancies were the top three types of malignancy. The risk factors for malignancy included old age (≥ 45 years old) and low serum levels of creatine phosphokinase (CPK) for patients with DM and male sex and low serum levels of CPK for patients with PM. Low serum levels of CPK in patients with myositis with malignancy represented a low degree of muscle destruction/inflammation, which might be attributed to activation of the PD-L1 pathway by tumor cells, thus inducing T-cell dysfunction mediating immune responses in myofibers. A treatment and follow-up algorithm should explore the occurrence of malignancy in different tissues and organs and suggested annual follow-ups for at least 5.5 years to cover the 80% cumulative incidence of malignancy in patients with DM and PM.
Topics: Adult; Aged; Aged, 80 and over; Comorbidity; Dermatomyositis; Diagnosis, Differential; Female; Humans; Incidence; Male; Middle Aged; Polymyositis; Public Health Surveillance; Registries; Risk Assessment; Risk Factors; Taiwan; Young Adult
PubMed: 33633147
DOI: 10.1038/s41598-021-83729-5