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Clinical and Experimental Rheumatology Feb 2024The 2017 EULAR/ACR classification criteria for adult/juvenile idiopathic inflammatory myopathies (IIM) were established using a data-driven approach by an international... (Review)
Review
The 2017 EULAR/ACR classification criteria for adult/juvenile idiopathic inflammatory myopathies (IIM) were established using a data-driven approach by an international group of myositis experts to allow classification of IIM and its major subtypes. Since their publication, the performance of the criteria has been tested in multiple cohorts worldwide and significant limitations have been identified. Moreover, the understanding and classification of IIM have evolved since 2017. This scoping review was undertaken as part of a large international project to revise the EULAR/ACR criteria and aims to i) summarise the evidence from the current literature on the performance characteristics of the 2017 EULAR/ACR classification criteria in various cohorts and IIM subtypes, and ii) delineate the factors that need to be considered in the revision of the classification criteria. A systematic search of Medline (via PubMed), Cumulative Index to Nursing and Allied Health Literature, and conference abstract archives was conducted independently by three investigators for studies on the EULAR/ACR criteria published between October 2017 and January 2023. This scoping review of 19 articles and 13 abstracts revealed overall good performance characteristics of the EULAR/ACR criteria for IIM, yet deficiencies in lack of inclusion of certain IIM subtypes, such as immune mediated necrotising myopathy, amyopathic dermatomyositis, antisynthetase syndrome and overlap myositis. Published modifications that may improve the performance characteristics of the criteria for classification of IIM subtypes were also summarised. The results of this review suggest that a revision of the EULAR/ACR criteria is warranted.
Topics: Adult; Humans; Myositis; Dermatomyositis; Autoimmune Diseases
PubMed: 38436279
DOI: 10.55563/clinexprheumatol/vuc5py -
Cureus Jan 2024Dermatomyositis, an autoimmune inflammatory myositis commonly linked to polymyositis, is marked by inflammatory and degenerative transformations impacting muscles, skin,...
Dermatomyositis, an autoimmune inflammatory myositis commonly linked to polymyositis, is marked by inflammatory and degenerative transformations impacting muscles, skin, limb girdles, the neck, and the pharynx. These changes result in symmetrical weakness and diverse levels of muscle atrophy. Uncommonly, the condition may impact the esophagus, lungs, and heart. While dermatomyositis is believed to involve genetic, immunological, and environmental factors, its precise etiology remains elusive. Typically, the classical presentation involves a symmetrical proximal myopathy alongside dermatological manifestations such as a purplish-red rash affecting the face, arms, hands, legs, and other areas. Additional symptoms may include dysphagia, myalgia, fever, and weight loss. The primary objectives of managing dermatomyositis are to address muscular weakness, skin manifestations, and any underlying health concerns. Integral to this management is the utilization of physical therapy and rehabilitation interventions. This study introduces a 23-year-old female patient with a noteworthy medical history covering a duration of two months. The patient reported a chief complaint of persistent thigh pain and a concurrent complaint concerning bilateral weakness in upper and lower extremities. Furthermore, the patient faced the additional challenge of difficulty swallowing. Intriguingly, the patient's clinical presentation was marked not only by the aforementioned symptoms but also by the development of a distinctive facial rash. This facial rash was accompanied by symptoms of stiffness in both small and large joints and a reduction in the range of affected joints. The physiotherapeutic assessment revealed quadriparesis of bilateral upper and lower limbs. The rehabilitation programme for the patient was planned by targeting proprioceptors to increase dynamic trunk balance in patients with DM. The Proprioceptive Neuromuscular Facilitation (PNF) technique employs diagonal movement patterns, thereby proving instrumental in enhancing the patient's daily activities. This methodology serves to optimize the individual's capacity to execute routine daily tasks, promoting independence in their daily life. An investigation like the Nerve Conduction Velocity (NCV) report shows the absence of motor excitation, suggesting motor axonal neuropathy. This approach, comprising isometric, concentric, and eccentric contraction exercises, demonstrated efficacy in mitigating muscular weakness, enhancing motor function, and alleviating the diverse symptoms associated with this condition.
PubMed: 38410335
DOI: 10.7759/cureus.52873 -
International Journal of Molecular... Feb 2024Extracellular vesicles (EVs) are lipid-bilayer particles secreted from cells that primarily assist in cell-to-cell communication through the content of their cargo, such... (Review)
Review
Extracellular vesicles (EVs) are lipid-bilayer particles secreted from cells that primarily assist in cell-to-cell communication through the content of their cargo, such as proteins and RNA. EVs have been implicated in the pathogenesis of various autoimmune diseases, including dermatomyositis (DM), an inflammatory autoimmune disease characterized by distinct cutaneous manifestations, myopathy, and lung disease. We sought to review the role of EVs in DM and understand how they contribute to the pathogenesis and clinical characterization of the disease. We summarized the research progress on EVs in dermatomyositis based on recent publications. EV cargoes, such as double-stranded DNA, microRNA, and proteins, contribute to DM pathogenesis and mediate the proinflammatory response and cytokine release through signaling pathways such as the stimulator of interferon genes (STING) pathway. These nucleic acids and proteins have been proposed as disease-specific, stable biomarkers to monitor disease activity and responses to therapy. They also correlate with clinical parameters, inflammatory markers, and disease severity scores. Furthermore, some markers show an association with morbidities of DM, such as muscle weakness and interstitial lung disease. The continued study of EVs will help us to further elucidate our understanding of dermatomyositis.
Topics: Humans; Dermatomyositis; Extracellular Vesicles; MicroRNAs; Lung Diseases, Interstitial; Nucleic Acids; Proteins; Exosomes
PubMed: 38396646
DOI: 10.3390/ijms25041967 -
CNS Neuroscience & Therapeutics Feb 2024This study was designed to evaluate the magnetic resonance imaging (MRI) patterns of the lower limb muscles in dermatomyositis (DM) with anti-transcriptional... (Observational Study)
Observational Study
AIMS
This study was designed to evaluate the magnetic resonance imaging (MRI) patterns of the lower limb muscles in dermatomyositis (DM) with anti-transcriptional intermediate factor 1-γ (anti-TIF1-γ) antibody.
METHODS
This retrospective, observational, cross-sectional study enrolled 12 adult DM patients with anti-TIF1-γ antibody. Muscles were assessed for fascial edema, subcutaneous-tissue edema, muscle edema, and fatty replacement. These features were analyzed in relation to clinical characteristics.
RESULTS
All 12 patients underwent hip and thigh MRI, and 8 completed calf MRI. All patients showed myofascial edema, muscle edema, and fatty replacement, and 8 out of 12 further exhibited subcutaneous-tissue edema. Specifically, myofascial edema of the gastrocnemius was observed in all patients (8/8). The vastus intermedialis and vastus lateralis muscles showed the most severe muscle edema, whereas the caput breve of the biceps femoris, semitendinosus, and soleus muscles exhibited the most severe fatty replacement. Although only 1 patient exhibited asymmetric muscle weakness, 9 showed asymmetric muscle edema, and 10 showed asymmetric fatty replacement. Changes in muscle edema positively correlated with creatine kinase (CK) levels.
CONCLUSIONS
Myofascial edema of gastrocnemius was a prominent characteristic of anti-TIF1-γ-positive DM. Early detection of muscle edema, as well as CK levels, may be helpful for monitoring disease activity.
Topics: Adult; Humans; Dermatomyositis; Retrospective Studies; Cross-Sectional Studies; Muscle, Skeletal; Magnetic Resonance Imaging; Edema
PubMed: 38385674
DOI: 10.1111/cns.14647 -
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 -
Clinical and Experimental Rheumatology Feb 2024
Topics: Humans; Myositis; Dermatomyositis; Longitudinal Studies; Autoantibodies
PubMed: 38372711
DOI: 10.55563/clinexprheumatol/8u8p8x -
Clinical and Experimental Medicine Feb 2024Melanoma differentiation-associated protein 5 (MDA5) antibody positive amyopathic dermatomyositis (DM) is a rare inflammatory disease. So far, there is no official...
Melanoma differentiation-associated protein 5 (MDA5) antibody positive amyopathic dermatomyositis (DM) is a rare inflammatory disease. So far, there is no official treatment guideline in MDA5 amyopathic dermatomyositis, but early and aggressive immunosuppressive combination treatment can induce a stable remission. We retrospectively analyzed a cohort of eight patients (male n = 5) that were diagnosed with MDA5-positive amyopathic DM. Patient data comprised demographics, CT-guided diagnosis of pulmonary involvement, pulmonary function testing including forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) data on baseline and mean long-term follow-up of 51 months (24-92 months) to evaluate treatment strategies. Depending on severity of organ involvement treatments were individualized including cyclophosphamide, immunoglobulins and plasmapheresis. Simultaneously, oral treatment with tacrolimus was commenced in four of the eight patients. Most patients received remission maintenance therapy with a combination of tacrolimus, rituximab and low dose steroids. In all patients, improvement in FVC was recorded and five patients achieved an improvement in DLCO. An improvement in the CT imaging morphological findings was observed in four patients. Awareness for the entirety of all clinical and disease-related findings of amyopathic DM is crucial, and remission maintenance is often achieved with a combination of tacrolimus and rituximab.
Topics: Humans; Male; Female; Retrospective Studies; Tacrolimus; Rituximab; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Autoantibodies; Dermatomyositis
PubMed: 38367089
DOI: 10.1007/s10238-024-01300-8 -
Frontiers in Immunology 2024The prognosis of anti-melanoma differentiation-associated gene 5 positive dermatomyositis (anti-MDA5DM) is poor and heterogeneous. Rapidly progressive interstitial lung...
BACKGROUND
The prognosis of anti-melanoma differentiation-associated gene 5 positive dermatomyositis (anti-MDA5DM) is poor and heterogeneous. Rapidly progressive interstitial lung disease (RP-ILD) is these patients' leading cause of death. We sought to develop prediction models for RP-ILD risk in anti-MDA5DM patients.
METHODS
Patients with anti-MDA5DM were enrolled in two cohorts: 170 patients from the southern region of Jiangsu province (discovery cohort) and 85 patients from the northern region of Jiangsu province (validation cohort). Cox proportional hazards models were used to identify risk factors of RP-ILD. RP-ILD risk prediction models were developed and validated by testing every independent prognostic risk factor derived from the Cox model.
RESULTS
There are no significant differences in baseline clinical parameters and prognosis between discovery and validation cohorts. Among all 255 anti-MDA5DM patients, with a median follow-up of 12 months, the incidence of RP-ILD was 36.86%. Using the discovery cohort, four variables were included in the final risk prediction model for RP-ILD: C-reactive protein (CRP) levels, anti-Ro52 antibody positivity, short disease duration, and male sex. A point scoring system was used to classify anti-MDA5DM patients into moderate, high, and very high risk of RP-ILD. After one-year follow-up, the incidence of RP-ILD in the very high risk group was 71.3% and 85.71%, significantly higher than those in the high-risk group (35.19%, 41.69%) and moderate-risk group (9.54%, 6.67%) in both cohorts.
CONCLUSIONS
The CROSS model is an easy-to-use prediction classification system for RP-ILD risk in anti-MDA5DM patients. It has great application prospect in disease management.
Topics: Humans; Male; Dermatomyositis; Lung Diseases, Interstitial; Interferon-Induced Helicase, IFIH1; Retrospective Studies; Autoantibodies
PubMed: 38361940
DOI: 10.3389/fimmu.2024.1286973 -
Neuromuscular Disorders : NMD Mar 2024Anti-MDA5-positive dermatomyositis (MDA5-DM) often presents with extramuscular, especially pulmonary and skin manifestations, and apparent clinical signs of frank...
Anti-MDA5-positive dermatomyositis (MDA5-DM) often presents with extramuscular, especially pulmonary and skin manifestations, and apparent clinical signs of frank myositis can be missing (so called amyopathic DM). We hereby present two male patients who died from respiratory failure during the course of MDA5-DM. While overt signs of myositis or any skin involvement were absent at admission to hospital we noticed conspicuous inflammatory alterations in various skeletal muscles morphologically, showing different degrees of affection. Furthermore, pathological changes of the lungs compatible with rapid progressive interstitial lung disease and characteristic cutaneous vasculoocclusive features were identified at autopsy. This observation shows that muscles and skin are subclinically affected in a widespread fashion, hence subtle signs of muscle involvement should be sought after in anti-MDA5-positive patients with predominant lung affection to ensure adequate treatment.
Topics: Humans; Male; Dermatomyositis; Myositis; Muscle, Skeletal; Autopsy; Lung; Autoantibodies; Interferon-Induced Helicase, IFIH1
PubMed: 38354588
DOI: 10.1016/j.nmd.2024.01.009 -
Thoracic Cancer Mar 2024Cancer-associated dermatomyositis (CAD), a paraneoplastic syndrome characterized by dermatomyositis (DM), frequently presents in association with small cell lung cancer...
Cancer-associated dermatomyositis (CAD), a paraneoplastic syndrome characterized by dermatomyositis (DM), frequently presents in association with small cell lung cancer (SCLC). Although the advent of immune checkpoint inhibitors (ICIs) has revolutionized cancer treatment, their efficacy and safety in patients with concurrent autoimmune diseases (AD) and malignancies remains uncertain. Several studies have suggested the safe administration of ICIs in patients with AD, indicating that successful cancer therapy can alleviate CAD symptoms. Conversely, other studies have raised concerns about the potential for ICIs to exacerbate AD flares or immune-related adverse events (irAEs). A comparative analysis of two cases from our institution emphasizes the variability in ICI responses among SCLC patients with CAD. One patient, previously reported as a case study, exhibited significant clinical improvement in DM symptoms after ICI administration, whereas the other developed severe exfoliative skin changes and experienced an unfavorable prognosis. This variability emphasizes the need for careful patient selection and close monitoring during ICI treatment. We hypothesized that overweight or obese individuals and those with severe initial skin lesions and elevated lactate dehydrogenase levels are more susceptible to developing irAEs following ICI therapy. Therefore, caution is advised when considering immunotherapy in these patients.
Topics: Humans; Lung Neoplasms; Small Cell Lung Carcinoma; Dermatomyositis; Antineoplastic Agents, Immunological; Retrospective Studies; Autoimmune Diseases; Immunotherapy
PubMed: 38352989
DOI: 10.1111/1759-7714.15238