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Medicina (Kaunas, Lithuania) Nov 2022: Polymyositis and dermatomyositis (PM/DM) are classified as polygenic autoimmune diseases, whereas inflammatory bowel disease (IBD) is considered a polygenic...
: Polymyositis and dermatomyositis (PM/DM) are classified as polygenic autoimmune diseases, whereas inflammatory bowel disease (IBD) is considered a polygenic autoinflammatory disease. In the literature, several cases exist reporting the co-occurrence of both conditions. At the molecular level, PM/DM and IBD share common genetic determinants including interferon regulatory factor and vitamin D receptor susceptibility loci. Accumulating evidence underline several indicators that confer poor prognosis in IBD, including antinuclear antibody positivity and the presence of other autoimmune diseases, therefore the aim of this study is to assess the association between these entities. : This is a population-based retrospective study using data retrieved from a large electronic medical record in Israel, the Clalit health registry. The sample included PM/DM patients and age- and sex-frequency matched controls. The prevalence of IBD in PM/DM was compared between the two groups and logistic regression was applied to control for confounding variables. Predictors of IBD in patients with PM/DM were also explored. : Our study included 12,278 subjects with 2085 PM/DM patients and 10,193 age- and sex- frequency-matched controls. The incidence of IBD in patients with PM/DM was significantly higher even after controlling for various confounding variables (OR of 1.73, 95% CI 1.05-2.86, -value = 0.033). Anti-nuclear antibodies (ANA) positivity was found to be an independent predictor for IBD diagnosis in patients with PM/DM (OR 3.67, 95% CI 1.01-13.36, = 0.048). : Our analysis reports an association between IBD and PM/DM. Such association could point towards a common pathophysiological background. Further research is needed to further describe the clinical courses and whether a unique therapeutic approach is warranted.
Topics: Humans; Dermatomyositis; Retrospective Studies; Polymyositis; Autoimmune Diseases; Inflammatory Bowel Diseases
PubMed: 36556929
DOI: 10.3390/medicina58121727 -
Immunity, Inflammation and Disease Feb 2024The main subtypes of idiopathic inflammatory myopathies (IIMs)-polymyositis (PM) and dermatomyositis (DM)-are often presented as interstitial lung disease (ILD) in... (Review)
Review
A bibliometric analysis of the research status and trends in studies on polymyositis and dermatomyositis with interstitial lung disease from 2000 to 2022 using Web of Science.
BACKGROUND
The main subtypes of idiopathic inflammatory myopathies (IIMs)-polymyositis (PM) and dermatomyositis (DM)-are often presented as interstitial lung disease (ILD) in clinical practice; therefore, many researchers have combined the three studies into PM/DM with ILD.
METHODS
Using bibliometrics, the research status, progress, and hotspots of PM/DM with ILD between 2000 and 2022 were studied. Literature data on PM/DM with ILD were retrieved from the Web of Science (WoS) database for the research period. Visualization software, including VOSviewer, Pajek, CiteSpace, and Scimago Graphica were used for bibliometric analysis.
RESULTS
A total of 1555 relevant articles were obtained, and the overall research in this field showed an increasing trend. Regarding contributing countries and venues, Japan published the most articles while Rheumatology was the most prolific journal. Regarding authors, the most published article was by Wang Guochun from Changchun University of Technology in China. Keyword analysis and cocited literature cluster analysis showed that diagnosis, classification, autoantibodies, antibodies, prognosis, complications, and treatment of PM/DM with ILD have been hot topics in this field recently. Moreover, our study shows that anti-mda5 antibody, mortality, gene 5 antibody, IIMs, double-blind, and prognostic factors, among others, may be new hot topics.
CONCLUSION
This study found that research on PM/DM with ILD has increased over time, and scholars are paying more attention to this field. The development of new drugs for the management, treatment, and prevention of PM/DM with ILD is the primary task of researchers and a direction for future research in this field.
Topics: Humans; Dermatomyositis; Retrospective Studies; Polymyositis; Lung Diseases, Interstitial; Prognosis; Randomized Controlled Trials as Topic
PubMed: 38376948
DOI: 10.1002/iid3.1190 -
Internal Medicine Journal Oct 2022
Topics: Humans; Troponin; COVID-19 Vaccines; RNA, Messenger; COVID-19; Polymyositis; Vaccination; Myocarditis
PubMed: 36000405
DOI: 10.1111/imj.15882 -
RMD Open Aug 2023To evaluate the efficacy and safety of ustekinumab (UST) in a multicentre, randomised, double-blind, placebo-controlled trial in adult Japanese patients with active... (Randomized Controlled Trial)
Randomized Controlled Trial
Phase 3, multicentre, randomised, double-blind, placebo-controlled, parallel-group study of ustekinumab in Japanese patients with active polymyositis and dermatomyositis who have not adequately responded to one or more standard-of-care treatments.
OBJECTIVES
To evaluate the efficacy and safety of ustekinumab (UST) in a multicentre, randomised, double-blind, placebo-controlled trial in adult Japanese patients with active polymyositis (PM) and dermatomyositis (DM).
METHODS
Fifty-one Japanese adults diagnosed with active PM/DM who did not respond adequately to one or more standard-of-care treatments were randomised 1:1 to receive UST (n=25) or placebo (n=26). Participants received body weight-range based intravenous administration of UST (6 mg/kg) or placebo at week 0 followed by 90 mg subcutaneous (SC) administration of UST or placebo every 8 weeks from week 8 to week 24. At week 24, placebo group crossed over to receive body weight-range based intravenous administration of UST, and thereafter, all participants received/were to receive SC administration of UST 90 mg every 8 weeks (week 32 through to week 72). The primary efficacy endpoint was the proportion of participants who achieved minimal improvement (≥20) in the International Myositis Assessment and Clinical Studies Total Improvement Score (IMACS TIS) at week 24.
RESULTS
No statistically significant difference was seen in the proportion of participants who achieved minimal improvement (≥20) in IMACS TIS at week 24 between the treatment groups (UST 64.0% vs placebo 61.5%, p=0.94) based on the primary estimand of the primary endpoint analysis.
CONCLUSIONS
UST was safe and well tolerated but did not meet the primary efficacy endpoint in adult Japanese participants with active PM/DM based on the primary analysis at week 24 in the study.
TRIAL REGISTRATION NUMBER
NCT03981744.
Topics: Adult; Humans; Body Weight; Dermatomyositis; East Asian People; Polymyositis; Ustekinumab
PubMed: 37652554
DOI: 10.1136/rmdopen-2023-003268 -
Neurology India 2021Polymyositis is a group of muscle disease characterised by progressive muscle inflammation and predominantly muscle weakness. It usually presents subacutely with...
Polymyositis is a group of muscle disease characterised by progressive muscle inflammation and predominantly muscle weakness. It usually presents subacutely with proximal weakness and mild diffuse muscular pain. Some patients have atypical presentation like early respiratory difficulty, Motor neuron disease (MND), or isolated dysphagia which leads to delay in diagnosis and treatment. We present one such case.
Topics: Deglutition Disorders; Humans; Motor Neuron Disease; Muscle Weakness; Myositis; Polymyositis
PubMed: 34747820
DOI: 10.4103/0028-3886.329582 -
Neurology(R) Neuroimmunology &... Jul 2023Muscle microangiopathy due to dysfunction of endothelial cells because of inflammation is a critical hallmark of dermatomyositis (DM); however, its pathomechanism...
BACKGROUND AND OBJECTIVES
Muscle microangiopathy due to dysfunction of endothelial cells because of inflammation is a critical hallmark of dermatomyositis (DM); however, its pathomechanism remains unclear. The aim of this study was to evaluate the effect of immunogloblin G (IgG) from patients with idiopathic inflammatory myopathies (IIM) on muscle endothelial cells in vitro.
METHODS
Using a high-content imaging system, we analyzed whether IgG purified from sera from patients with IIM (n = 15), disease controls (DCs: n = 7), and healthy controls (HCs: n = 7) can bind to muscle endothelial cells and induce complement-dependent cellular cytotoxicity.
RESULTS
IgGs from Jo-1 antibody myositis could bind to muscle endothelial cells and caused complement-dependent cell cytotoxicity. RNA-seq demonstrated the upregulation of genes associated with tumor necrosis factor (TNF)-α, triggering receptor expressed on myeloid cells-1 (TREM-1), CD25, and mitochondria pathways after exposure to IgG from the Jo-1, signal recognition particle (SRP), and polymyositis (PM) groups. The high-content imaging system showed that TREM-1 expression in the Jo-1, SRP, and PM groups was increased in comparison with DCs and HCs and that the TNF-α expression in the Jo-1 group was higher in comparison with the SRP, PM, DC, and HC groups. The expression of TREM-1 was observed in biopsied capillaries and the muscle membrane from patients with Jo-1 and in biopsied muscle fiber and capillaries from patients with DM and SRP. The depletion of Jo-1 antibodies by IgG of patients with Jo-1 antibody myositis reduced the Jo-1 antibody-induced complement-dependent cellular cytotoxicity in muscle endothelial cells.
DISCUSSION
Jo-1 antibodies from Jo-1 antibody myositis show complement-dependent cellular cytotoxicity in muscle endothelial cells. IgGs from patients with Jo-1, SRP, and DM increase the TREM-1 expression in endothelial cells and muscles.
Topics: Humans; Triggering Receptor Expressed on Myeloid Cells-1; Endothelial Cells; Up-Regulation; Myositis; Polymyositis; Muscles; Immunoglobulin G
PubMed: 37147138
DOI: 10.1212/NXI.0000000000200116 -
Revista Brasileira de Reumatologia 2015Currently, there are few studies that describe pregnancy in dermatomyositis/polymyositis patients, and they are largely limited to case reports or studies with few...
BACKGROUND
Currently, there are few studies that describe pregnancy in dermatomyositis/polymyositis patients, and they are largely limited to case reports or studies with few samples.
OBJECTIVES
Therefore, we describe the pregnancy in a large sample of patients with dermatomyositis/polymyositis and to analyze the outcomes in those who became pregnant during or after disease onset.
METHODS
The present single-center study analyzed 98 female patients with idiopathic inflammatory myopathies (60 dermatomyositis and 38 polymyositis patients). They were interviewed to obtain obstetric antecedent and demographic data from June 2011 to June 2012.
RESULTS
Seventy-eight (79.6%) of the 98 patients had obstetric histories. Six polymyositis and 9 dermatomyositis patients became pregnant after disease onset. The pregnancy outcomes in these cases were good, except in the following cases: 1 disease reactivation, 1 intrauterine growth retardation, 1 diabetes mellitus, 1 hypertension, 1 hypothyroidism, and 2 fetal losses (same patient). Moreover, 2 patients developed dermatomyositis during pregnancy and 4 (2 polymyositis and 2 dermatomyositis) during the postpartum period with good control after glucocorticoid and immunosuppressant therapy.
CONCLUSIONS
The adverse obstetric events were related to clinical intercurrences and the pregnancy does not seem to carry a worse prognosis specifically in disease (for example: disease relapsing). Moreover, dermatomyositis or polymyositis onset during pregnancy or the postpartum period had good outcome after drug therapy.
Topics: Adult; Cohort Studies; Dermatomyositis; Female; Humans; Polymyositis; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Retrospective Studies; Young Adult
PubMed: 25577487
DOI: 10.1016/j.rbr.2014.10.001 -
Seminars in Arthritis and Rheumatism Dec 2018Cigarette smoking is associated with immune-mediated disorders. We explored the contribution of smoking to polymyositis (PM) and dermatomyositis (DM) phenotypes and...
OBJECTIVE
Cigarette smoking is associated with immune-mediated disorders. We explored the contribution of smoking to polymyositis (PM) and dermatomyositis (DM) phenotypes and attempted to determine whether cigarette smoking effects differ by race and genotype.
METHODS
Associations of tobacco smoking with disease features, autoantibodies, HLA types, and race were evaluated using multiple logistic regressions in 465 patients.
RESULTS
Caucasian ever-smokers (n = 140) were more likely to have PM (adjusted OR = 2.24, 95% CI: 1.41\x963.57), anti-synthetase (adjusted OR = 1.93, 95% CI: 1.12\x963.34) and anti-Jo-1 autoantibodies (adjusted OR = 1.94, 95% CI: 1.08\x963.46) and less likely to have anti-p155/140 autoantibodies (adjusted OR = 0.36, 95% CI: 0.14\x960.92). In Caucasians, ever-smokers had a greater interstitial lung disease (ILD) frequency than never-smokers, while in African-Americans this relationship was inverted, but neither trend reached statistical significance. Pack-years of cigarette smoking showed significant positive associations with PM (adjusted OR = 1.02, 95% CI: 1.002\x961.04) and ILD (adjusted OR = 1.02, 95% CI: 1.001\x961.03) and was inversely associated with anti-p155/140 autoantibodies (adjusted OR = 0.93, 95% CI: 0.87\x960.99) in Caucasians. Caucasian heavy smokers (=20 pack-years) were more likely to have PM (adjusted OR = 2.52, 95% CI: 1.25\x965.09), ILD (adjusted OR = 2.48, 95% CI: 1.23\x965.00) and anti-Jo-1 autoantibodies (adjusted OR = 2.65, 95% CI: 1.16\x966.08) than never-smokers. In Caucasians, compared to never-smokers without HLA-DRB1*03:01 allele, ever-smokers with HLA-DRB1*03:01 allele had the highest odds of PM, ILD, ASA, and anti-Jo-1 autoantibodies. Risks for those with only one of these two factors were intermediate. An inverse pattern was observed regarding anti-p155/140 autoantibodies.
CONCLUSION
Tobacco smoking was associated with clinical and autoantibody phenotypes in Caucasians. Our findings also suggest possible interactions among HLA-DRB1*03:01 and smoking on the risk of PM and ILD, as well as, anti-synthetase, anti-Jo-1, and anti-p155/140 autoantibodies in Caucasians.
Topics: Adult; Autoantibodies; Cigarette Smoking; Dermatomyositis; Female; Humans; Male; Middle Aged; Phenotype; Polymyositis
PubMed: 29703532
DOI: 10.1016/j.semarthrit.2018.02.003 -
Contrast Media & Molecular Imaging 2022This paper aims to investigate the clinical and laboratory test characteristics of patients with anti-MDA5 antibody-positive PM/DM by analyzing the clinical...
This paper aims to investigate the clinical and laboratory test characteristics of patients with anti-MDA5 antibody-positive PM/DM by analyzing the clinical characteristics, laboratory test results, and 1-year survival rate of patients with anti-MDA5 antibody-positive PM/DM in polymyositis (PM) and dermatomyositis (DM). To further investigate the impact of positive anti-MDA5 antibodies on the prognosis of PM/DM patients. According to the anti-MDA5 antibody test results, 18 cases with positive anti-MDA5 antibodies were in the positive group and 46 cases with negative anti-MDA5 antibodies were in the negative group. The clinical manifestations, laboratory tests, treatment protocols, and prognostic risk factors were collected for both groups. The chi-square test, Mann-Whitney method, Fisher test, -test, Kaplan-Meier method, and Log-rank test were used for statistical analysis. Anti-MDA5 antibody positivity was more common in patients with DM/CADM. With no statistically significant differences in age and sex ratio between the two groups, The differences in erythrocyte sedimentation rate (ESR), ferritin (Fer), and creatine kinase (CK) levels in the positive group were statistically significant compared with the negative group. Clinically, the positive group was more prone to arthralgia, skin rash, and interstitial pneumonia.
Topics: Autoantibodies; Dermatomyositis; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Polymyositis
PubMed: 35992549
DOI: 10.1155/2022/7102480 -
Seminars in Arthritis and Rheumatism Feb 2020Juvenile idiopathic inflammatory myopathies (JIIM) are rare, chronic autoimmune muscle diseases of childhood, with the potential for significant morbidity. Data on...
OBJECTIVE
Juvenile idiopathic inflammatory myopathies (JIIM) are rare, chronic autoimmune muscle diseases of childhood, with the potential for significant morbidity. Data on long-term outcomes is limited. In this study we investigate correlations between clinical and demographic features with long-term outcomes in a referral population of adult patients with JIIM.
METHODS
Forty-nine adults with JIIM were assessed at two referral centers between 1994 and 2016. Features of active disease and damage at a cross-sectional assessment were obtained. Regression modeling was used to examine factors associated with long-term outcomes, defined by the presence of calcinosis or a higher adjusted Myositis Damage Index (MDI) score. A multivariable model of MDI was constructed using factors that were statistically significant in bivariate models.
RESULTS
At a median of 11.5 [IQR 4.5-18.9] years following diagnosis, median American College of Rheumatology (ACR) functional class was 2 [1.5-3.0], Health Assessment Questionnaire (HAQ) score was 0.4 out of 3.0 [0.0-1.0], and manual muscle testing (MMT) score was 229 out of 260 [212.6-256.8]. Median MDI score was 6.0 [3.5-8.9], with the most commonly damaged organ systems being cutaneous and musculoskeletal. Factors associated with an elevated MDI score were the presence of erythroderma and other cutaneous manifestations, disease duration, and ACR functional class. Calcinosis was present in 55% of patients. The strongest predictors of calcinosis were disease duration, periungual capillary changes, and younger age at diagnosis.
CONCLUSION
In a tertiary referral population, long-term functional outcomes of JIIM are generally favorable, with HAQ scores indicative of mild disability. Although most patients had mild disease activity and virtually all had significant disease damage, severe or systemic damage was rare. Certain clinical features are associated with long-term damage and calcinosis.
Topics: Adolescent; Adult; Calcinosis; Dermatomyositis; Female; Humans; Male; Middle Aged; Severity of Illness Index; Skin; Treatment Outcome; Young Adult
PubMed: 31303436
DOI: 10.1016/j.semarthrit.2019.06.014