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The Netherlands Journal of Medicine Oct 2011Polymyositis (PM) and dermatomyositis (DM) are rare idiopathic inflammatory myopathies (IIM) with a presumed autoimmune pathogenesis. Typical features are subacute... (Review)
Review
Polymyositis (PM) and dermatomyositis (DM) are rare idiopathic inflammatory myopathies (IIM) with a presumed autoimmune pathogenesis. Typical features are subacute onset, proximal, symmetric muscle weakness, elevated serum creatine kinase, and mononuclear cell infiltrates in the muscle biopsy. Strong support for an autoimmune pathogenesis comes from histopathological findings in biopsies of affected muscles. Furthermore, the association with autoantibodies supports the notion that immune-mediated inflammation is involved. PM and DM may occur in isolation or in connection with a connective tissue disease or cancer. The current treatment for IIM consists of first-line high-dose steroids and various conventional second-line treatments. Improvements in treatment for IIM are hampered by difficulties in the design of trials and the low incidence and prevalence of the disease. Cytokines and chemokines are factors involved in the inflammatory process in IIM, and are candidates for future therapeutic targets. Preliminary data with anti-tumour necrosis factor therapy are not very promising, but results of blockers of the lymphotoxin signalling pathway are to be awaited. Anti-B cell therapy may be a valuable therapeutic option for treatment of refractory IIM. The effects of anti-interferon-alpha in IIM are to be awaited, as are results of other anti-cytokine therapies and anti-chemokine therapy. Outcome measures to be used in clinical trials in II M include at present the core sets of outcome proposed by the International Myositis Assessment Clinical Study Group (IMACS).
Topics: Antibodies, Monoclonal, Murine-Derived; Dermatomyositis; Glucocorticoids; Humans; Immunologic Factors; Immunosuppressive Agents; Interferon-alpha; Polymyositis; Rituximab; Tumor Necrosis Factor Inhibitors
PubMed: 22058260
DOI: No ID Found -
The Western Journal of Medicine Apr 1976
Topics: Dermatomyositis; Humans; Male; Methotrexate; Middle Aged; Steroids
PubMed: 1266216
DOI: No ID Found -
The Journal of Investigative Dermatology Dec 2023In the past decade, there have been six industry-sponsored phase 3 trials in adult patients with dermatomyositis (DM), primarily focusing on improving muscle weakness....
Validation of Cutaneous Dermatomyositis Disease Area and Severity Index Activity Score and Other Efficacy Outcomes as Measures of Skin Disease in Dermatomyositis in the Lenabasum Phase 3 Trial.
In the past decade, there have been six industry-sponsored phase 3 trials in adult patients with dermatomyositis (DM), primarily focusing on improving muscle weakness. However, skin disease is a cardinal manifestation of DM. This study evaluated the sensitivity of Cutaneous Dermatomyositis Disease Area and Severity Index Activity score, Cutaneous Dermatomyositis Activity Investigator Global Assessment, Total Improvement Score, and other outcome measures used in DM clinical trials to detect improvement in DM skin disease activity. Data analyzed from the lenabasum phase 3 trial in DM showed that improvement in Cutaneous Dermatomyositis Disease Area and Severity Index Activity score increased proportionately with the degree of patient- or physician-reported improvement in skin disease, consistently measuring improvement when clinically meaningful improvement was reported at weeks 16-52. In contrast, Cutaneous Dermatomyositis Activity Investigator Global Assessment measured little change from baseline with reported no improvement in skin disease but also a similar change from baseline with slight improvement. No Skindex-29+3 subscale performed well at reflecting increasing degrees of improvement in skin disease. Extramuscular Global Assessment and Total Improvement Score generally showed increasing levels of improvement as the degree of patient- and physician-reported improvement in skin disease increased, but these are composite measures and are not specific to improvement in DM skin disease. To measure clinically meaningful improvement in skin disease in a DM trial, Cutaneous Dermatomyositis Disease Area and Severity Index Activity score is the more sensitive outcome measure across time points.
Topics: Adult; Humans; Dermatomyositis; Severity of Illness Index; Skin; Outcome Assessment, Health Care
PubMed: 37331616
DOI: 10.1016/j.jid.2023.05.025 -
Journal of Investigative Medicine High... 2021Psoriatic arthritis is an inflammatory arthritis, most commonly occurring several years after the onset of psoriasis. Psoriatic arthritis is associated with many...
Psoriatic arthritis is an inflammatory arthritis, most commonly occurring several years after the onset of psoriasis. Psoriatic arthritis is associated with many comorbidities, including diabetes mellitus, nonalcoholic fatty liver disease, fibromyalgia, and cardiovascular disease. Dermatomyositis is an inflammatory myopathy primarily affecting the skin and muscles. As per literature review, cases of psoriasis and dermatomyositis have been reported. In most published cases, the courses of these diseases develop independently. This is the case of a 45-year-old woman initially diagnosed with psoriatic arthritis who developed concurrent dermatomyositis. The methods used were PubMed search and UpToDate search.
Topics: Arthritis, Psoriatic; Dermatomyositis; Humans; Middle Aged
PubMed: 34911389
DOI: 10.1177/23247096211057702 -
Clinical and Experimental Rheumatology Feb 2022Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are idiopathic inflammatory myopathies, which can be resistant and unresponsive to initial treatments, leading to...
OBJECTIVES
Dermatomyositis (DM) and juvenile dermatomyositis (JDM) are idiopathic inflammatory myopathies, which can be resistant and unresponsive to initial treatments, leading to severe complications and impaired quality of life. There are few randomised trials in dermatomyositis and the outcomes reported may not be consistent, which can limit decision-making. The aim of this study is to assess the scope and consistency of outcomes reported in randomised trials in dermatomyositis.
METHODS
MEDLINE, Embase, PsycINFO and clinicaltrials.gov were searched from 1993-2020 for randomised trials in children and adults with dermatomyositis. The frequency and characteristics of the outcomes reported were analysed and classified.
RESULTS
20 trials were included. Across these trials, a total of 743 outcome measures were reported, which were grouped into 34 outcome domains; of which 17 were clinical, 13 were surrogate/biochemical, and 4 were patient-reported outcomes. The top five most frequently reported outcome domains were muscle inflammation (15 trials, 46 outcome measures), physical function (14 trials, 16 outcome measures), muscle strength (13 trials, 30 outcome measures), global health (12 trials, 33 outcome measures) and immunologic marker (11 trials, 91 outcomes).
CONCLUSIONS
The majority of outcomes reported in trials in people with dermatomyositis and JDM are clinical and surrogate outcomes rather than patient-reported outcomes. The outcomes reported are very inconsistent across trials, with wide heterogeneity in the measures used. Standardised reporting of critically important outcomes is needed to strengthen the value of trials for decision-making.
Topics: Adult; Child; Dermatomyositis; Humans; Outcome Assessment, Health Care; Patient Reported Outcome Measures; Quality of Life; Randomized Controlled Trials as Topic
PubMed: 35225217
DOI: 10.55563/clinexprheumatol/3izscd -
Frontiers in Immunology 2018Juvenile Dermatomyositis (JDM) is a systemic immune-mediated disease of childhood, characterized by muscle weakness, and a typical skin rash. Other organ systems and... (Review)
Review
Juvenile Dermatomyositis (JDM) is a systemic immune-mediated disease of childhood, characterized by muscle weakness, and a typical skin rash. Other organ systems and tissues such as the lungs, heart, and intestines can be involved, but may be under-evaluated. The inflammatory process in JDM is characterized by an interferon signature and infiltration of immune cells such as T cells and plasmacytoid dendritic cells into the affected tissues. Vasculopathy due to loss and dysfunction of endothelial cells as a result of the inflammation is thought to underlie the symptoms in most organs and tissues. JDM is a heterogeneous disease, and several disease phenotypes, each with a varying combination of affected tissues and organs, are linked to the presence of myositis autoantibodies. These autoantibodies have therefore been extensively studied as biomarkers for the disease phenotype and its associated prognosis. Next to identifying the JDM phenotype, monitoring of disease activity and disease-inflicted damage not only in muscle and skin, but also in other organs and tissues, is an important part of clinical follow-up, as these are key determinants for the long-term outcomes of patients. Various monitoring tools are currently available, among which clinical assessment, histopathological investigation of muscle and skin biopsies, and laboratory testing of blood for specific biomarkers. These investigations also give novel insights into the underlying immunological processes that drive inflammation in JDM and suggest a strong link between the interferon signature and vasculopathy. New tools are being developed in the quest for minimally invasive, but sensitive and specific diagnostic methods that correlate well with clinical symptoms or reflect local, low-grade inflammation. In this review we will discuss the types of (extra)muscular tissue inflammation in JDM and their relation to vasculopathic changes, critically assess the available diagnostic methods including myositis autoantibodies and newly identified biomarkers, and reflect on the immunopathogenic implications of identified markers.
Topics: Autoantibodies; Biomarkers; Biopsy; Child; Dermatomyositis; Humans; Muscle, Skeletal; Prognosis; Skin; Vascular Diseases
PubMed: 30619311
DOI: 10.3389/fimmu.2018.02951 -
Journal of Proteome Research Jan 2023Dermatomyositis and polymyositis (DM/PM) are systemic autoimmune diseases characterized by proximal muscle weakness. The underlying pathogenetic mechanism of this...
Dermatomyositis and polymyositis (DM/PM) are systemic autoimmune diseases characterized by proximal muscle weakness. The underlying pathogenetic mechanism of this disease remains under-researched. Here, using proteomics analysis, a great overlap of differentially expressed plasma exosomal proteins involved in the complement and coagulation cascade pathway, including FGA, FGB, FGG, C1QB, C1QC, and VWF, was identified in DM/PM patients versus healthy controls. Correlation analysis showed that the expression levels of complement-associated proteins (C1QB and C1QC) correlated positively with CRP, ESR, and platelet count. ROC curve analysis demonstrated that complement and coagulation cascade-associated proteins could be strong predictors for DM/PM. In addition, we also identified several other proteins that were differentially expressed in DM and PM. The selected candidate proteins were further validated by parallel reaction monitoring (PRM) and enzyme-linked immunosorbent assay (ELISA). Together, our findings indicate that these exosome-derived proteins might participate in microvascular damage in DM/PM through the activation of the complement and coagulation cascade pathway and function as biomarkers for the clinical diagnosis of DM/PM.
Topics: Humans; Dermatomyositis; Exosomes; Proteomics; Polymyositis; Biomarkers; Complement System Proteins
PubMed: 36507906
DOI: 10.1021/acs.jproteome.2c00532 -
Acta Medica Portuguesa 2000Dermatomyositis (DM) is an inflammatory myopathy characterized by a typical cutaneous rash and proximal weakness. DM results from endothelium deposition of the...
Dermatomyositis (DM) is an inflammatory myopathy characterized by a typical cutaneous rash and proximal weakness. DM results from endothelium deposition of the complement membranolytic attack complex C5b-9, followed by inflammation. It is thus possible to have cases of DM with the typical rash, but with no associated myositis--amiopathic DM. DM represents a higher risk of association with malignancy and sometimes behaves as a paraneoplastic syndrome. As DM can be the alarm sign, once diagnosed, exclusion of malignancy should be done. In this article we also discuss the treatment and prognosis of dermatomyositis, according to the literature.
Topics: Adolescent; Adrenal Cortex Hormones; Adult; Creatine Kinase; Dermatomyositis; Electromyography; Humans; Paraneoplastic Syndromes; Prognosis; Skin
PubMed: 11234494
DOI: No ID Found -
BMJ Case Reports Nov 2020We provide the first report of amyopathic dermatomyositis combined with peripheral neuropathy. Our patient, a 49-year-old woman, initially experienced muscle weakness... (Review)
Review
We provide the first report of amyopathic dermatomyositis combined with peripheral neuropathy. Our patient, a 49-year-old woman, initially experienced muscle weakness and tingling sensations in her legs, and nerve conduction study findings and the detection of antiganglioside antibodies indicated that she had autoimmune peripheral neuropathy. The unexpected presence of skin lesions, interstitial pneumonia and antibodies to melanoma differentiation-associated protein 5 prompted an additional diagnosis of amyopathic dermatomyositis. No previous report has described amyopathic dermatomyositis with peripheral neuropathy, and the present case provides evidence for the once-controversial concept of neuromyositis.
Topics: Adolescent; Aged; Biopsy; Child; Dermatomyositis; Diagnosis, Differential; Female; Humans; Immunotherapy; Male; Middle Aged; Peripheral Nervous System Diseases; Skin; Tomography, X-Ray Computed
PubMed: 33257380
DOI: 10.1136/bcr-2020-237250 -
Rheumatology International Jun 2021Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with... (Review)
Review
Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with Rapidly Progressive Interstitial Lung Disease (RP-ILD) and unique cutaneous features. We reviewed PubMed, SCOPUS and Web of Science databases and selected 87 relevant articles after screening 1485 search results, aiming to gain a better understanding of the pathophysiology, clinical features, diagnosis, and treatment approaches of anti-MDA-5 DM described in the literature. The etiopathogenesis is speculatively linked to an unidentified viral trigger on the background of genetic predisposition culminating in an acquired type I interferonopathy. The clinical phenotype is highly varied in different ethnicities, with new clinical features having been recently described, expanding the spectrum of cases that should raise the suspicion of anti-MDA-5 DM. Unfortunately, the diagnosis is frequently missed despite excessive mortality, calling for wider awareness of suspect symptoms. RP ILD is the major determinant of survival, treatment being largely based on observational studies with recent insights into aggressive combined immunosuppression at the outset.
Topics: COVID-19; Dermatomyositis; Disease Progression; Exanthema; Female; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Male; Prevalence; SARS-CoV-2
PubMed: 33774723
DOI: 10.1007/s00296-021-04819-1