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Frontiers in Immunology 2023We aimed to identify B-cell-mediated immunomechanisms in inclusion body myositis (IBM) and polymyositis (PM) as part of the complex pathophysiology.
INTRODUCTION
We aimed to identify B-cell-mediated immunomechanisms in inclusion body myositis (IBM) and polymyositis (PM) as part of the complex pathophysiology.
MATERIALS AND METHODS
Human primary myotube cultures were derived from orthopedic surgery. Diagnostic biopsy specimens from patients with IBM (n=9) and PM (n=9) were analyzed for markers of B cell activation (BAFF and APRIL) and for chemokines that control the recruitment of B cells (CXCL-12 and CXCL-13). Results were compared to biopsy specimens without myopathic changes (n=9) and hereditary muscular dystrophy (n=9).
RESULTS
The mRNA expression of BAFF, APRIL, and CXCL-13 was significantly higher in IBM and PM compared to controls. Patients with IBM displayed the highest number of double positive muscle fibers for BAFF and CXCL-12 (48%) compared to PM (25%), muscular dystrophy (3%), and non-myopathic controls (0%). , exposure of human myotubes to pro-inflammatory cytokines led to a significant upregulation of BAFF and CXCL-12, but APRIL and CXCL-13 remained unchanged.
CONCLUSION
The results substantiate the hypothesis of an involvement of B cell-associated mechanisms in the pathophysiology of IBM and PM. Muscle fibers themselves seem to contribute to the recruitment of B cells and sustain inflammation.
Topics: Humans; Myositis; Polymyositis; Myositis, Inclusion Body; Inflammation; Muscle Fibers, Skeletal
PubMed: 37731487
DOI: 10.3389/fimmu.2023.1177721 -
American Journal of Reproductive... Nov 2022Idiopathic inflammatory myopathy (IIM) in pregnancy is uncommon but may result in complications for both mother and the fetus. (Review)
Review
BACKGROUND
Idiopathic inflammatory myopathy (IIM) in pregnancy is uncommon but may result in complications for both mother and the fetus.
AIM
In this systematic review, we summarized the current literature investigating outcomes of pregnancy related to the dermatomyositis/polymyositis (DM/PM) process.
CONTENT
We searched PubMed, Embase, Cochrane Library, and Web of Science databases and included 61 studies reporting the disease course, pregnancy outcomes, and management of both pregnancy and DM/PM in the final analysis.The specific information of 221 pregnancies was extracted and these pregnancies were divided into three distinct forms: pregnancies after disease onset (n = 159), pregnancies with new disease onset (n = 37), and pregnancies followed by postpartum onset (n = 25). In most cases, DM/PM disease activity remained stable or improved throughout pregnancy (80.2%) and the postpartum period (83.9%). Active DM/PM during pregnancy significantly increased the risk of stillbirth or neonatal death (12% vs. 1%, P = .005) and preterm birth (34.7% vs. 11%, P < .001). The rates of other poor outcomes (total fetal loss, low birth weight, and intrauterine growth retardation) were also increased in pregnancies with active disease. Mainstay treatments for active DM/PM during pregnancy are glucocorticoids and intravenous immunoglobins.
IMPLICATIONS
The present results underline the importance of good control of myopathy in optimizing the pregnancy outcomes of women with DM/PM.
Topics: Pregnancy; Adult; Infant, Newborn; Female; Humans; Dermatomyositis; Premature Birth; Polymyositis; Pregnancy Outcome; Glucocorticoids
PubMed: 35867856
DOI: 10.1111/aji.13603 -
International Journal of Rheumatic... Dec 2023
Topics: Humans; Dermatomyositis; Autoantibodies
PubMed: 37653610
DOI: 10.1111/1756-185X.14895 -
European Journal of Internal Medicine Apr 2023To evaluate the efficacy and safety of tacrolimus for dermatomyositis (DM) and polymyositis (PM) treatment. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To evaluate the efficacy and safety of tacrolimus for dermatomyositis (DM) and polymyositis (PM) treatment.
METHODS
We searched the Embase, PubMed, the Cochrane Central Register of Controlled Trials, and China National Knowledge Infrastructure were used as searching tools from inception up to October 2022. Two authors independently selected studies. The available studies were comprehensively reviewed and investigated.
RESULTS
A total of 9 studies, including 350 patients, were analysed. Pooled results showed a higher overall survival rate in tacrolimus therapy group. Creatine kinase (CK) levels and forced vital capacity (FVC) showed significant improvement after tacrolimus therapy. The incidence of adverse events including infection and renal dysfunction showed no significant differences between the tacrolimus therapy group and conventional therapy group.
CONCLUSION
The results of this meta-analysis indicated that GC therapy in combination with tacrolimus therapy could help improving overall survival rate, pulmonary function and had similar safety outcomes compared to conventional therapy in DM and PM patients.
Topics: Humans; Dermatomyositis; Drug Therapy, Combination; Immunosuppressive Agents; Polymyositis; Tacrolimus
PubMed: 36725399
DOI: 10.1016/j.ejim.2023.01.018 -
Rheumatology International Feb 2020The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases resulting from inflammation of muscle and manifesting as weakness, though a range of... (Review)
Review
The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases resulting from inflammation of muscle and manifesting as weakness, though a range of extra-muscular manifestations are observed. These are often correlated closely with disease subtype and the presence of myositis-specific/myositis-associated antibodies. IIM are notoriously difficult to treat and often refractory to glucocorticoid therapy and synthetic immunosuppressants. Both the innate and adaptive immune systems are implicated in the pathogenesis of IIM. A growing understanding of the key cytokines as well as the cell-mediated and antibody effectors of disease has identified multiple potential targets for biologic therapy. The most widely used of these is B-cell depletion via rituximab though the tumour necrosis factor inhibitors and other biologic therapies used in diseases such as rheumatoid arthritis, systemic lupus erythematosus and multiple sclerosis have also been trialled. This review summarises the literature thus far on biologic therapy in IIM, highlighting both the significant trials that influence current treatment regimens and also the continuing need for further research to inform more effective therapies.
Topics: Abatacept; Alemtuzumab; Antibodies, Monoclonal, Humanized; Antirheumatic Agents; Basiliximab; Biological Products; Dermatomyositis; Humans; Immunologic Factors; Immunosuppressive Agents; Myositis; Myositis, Inclusion Body; Polymyositis; Rituximab; Tumor Necrosis Factor Inhibitors
PubMed: 31680207
DOI: 10.1007/s00296-019-04467-6 -
Neurotherapeutics : the Journal of the... Apr 2022Inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), antisynthetase syndrome (ASS) and... (Review)
Review
Inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), antisynthetase syndrome (ASS) and overlap myositis (OM), in short myositis, are rare diseases. All forms of myositis have progressive muscle weakness in common, with each subtype characterized by different autoantibody profiles, histological findings and extramuscular manifestations. Due to better understanding of the pathogenesis of the muscle inflammation in myositis, new molecular pathways for targeted therapy have been discovered. Current therapies aim at different components of the innate or the adaptive immune response. Additionally, non-inflammatory mechanisms in myositis have come into focus as possible treatment targets. The use of therapeutical antibodies in myositis has been examined in various clinical studies, several of them randomized controlled ones: Depletion of B-cells by rituximab has been established as treatment of refractory myositis. IVIG, an antibody therapy in the wider sense, has now been licensed for DM following a recent positive clinical trial. Negative study results were reported in randomized trials with infliximab, sifalimumab and bimagrumab. Studies on basiliximab and eculizumab are currently underway, and are expected to yield results in a couple of years. Despite some promising results of clinical studies with antibody therapy in myositis, further research is crucial to optimize the treatment for this debilitating disease and to find treatment alternatives for treatment-refractory patients.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Myositis; Myositis, Inclusion Body; Polymyositis
PubMed: 35394612
DOI: 10.1007/s13311-022-01220-z -
Clinical Rheumatology Feb 2022
Topics: Connective Tissue Diseases; Humans; Myocarditis; Polymyositis; Scleroderma, Systemic; Syndrome
PubMed: 34518973
DOI: 10.1007/s10067-021-05917-y -
Autoimmunity Reviews Aug 2023Rapidly progressive interstitial lung disease (RP-ILD) clearly harms the prognoses of dermatomyositis/polymyositis (DM/PM) patients, however there is a dearth of... (Meta-Analysis)
Meta-Analysis Review
Rapidly progressive interstitial lung disease (RP-ILD) clearly harms the prognoses of dermatomyositis/polymyositis (DM/PM) patients, however there is a dearth of numerical prevalence and therapy comparison in this field. Therefore, the purpose of this study was to determine the prevalence of RP-ILD in DM/PM patients and compare prognoses, including remission rate and survival data, between treatments. Studies with reports of RP-ILD in DM/PM patients and studies with definite remission and/or survival data of DM/PM-RP-ILD were included in the study. Data sources were Pubmed, Embase, and Cochrane Library without language restrictions. Two authors (WHL and WWQ) extracted independently the data. Estimates of the pooled effects were calculated using the Mantel-Haenszel technique (random effects). The prevalence meta-analysis included 18 papers with 6058 DM/PM patients, and 31 papers were analyzed for treatment effects, including remission rate, 6-month survival rate, 1-year survival rate, and 5-year survival rate. Database search yielded 1816 articles. In the DM/PM population, the combined prevalence of RP-ILD was 8.9% (95% CI, 5.8% to 12.1%). Patients with RP-ILD have a remission rate of 58.4% (95% CI, 47.3% to 69.4%), with biologic treatment with the highest remission rate, followed by triple therapy (defined as adding a third intravenous medication, including cyclophosphamide and immunoglobulin). Biologics therapy had the highest overall survival rate at six months (95% CI, 49.8% to 73.9%), followed by cDMARDs, plasma exchange, and triple therapy. The 1-year survival rate was 77.4% (95% CI, 66.7% to 88.1%), and triple therapy and cDMARDs had the best survival rates. The 5-year survival rate was 40.0% (95% CI, 10.0% to 69.9%). The prevalence of RP-ILD in DM/PM was approximately 8.9%, with a poor long-term prognosis. The use of biological agents appears to provide the best therapeutic outcomes, providing RP-ILD management with a novel evidence-based therapy. The use of strong immunosuppressive treatments may result in life-threatening side effects, thus clinicians must closely monitor the condition.
Topics: Prevalence; Polymyositis; Humans; Adult; Dermatomyositis; Lung Diseases, Interstitial; Treatment Outcome
PubMed: 37164215
DOI: 10.1016/j.autrev.2023.103335 -
Expert Review of Clinical Immunology Feb 2023The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, the anti-synthetase syndromes, immune-mediated necrotizing myopathy and... (Review)
Review
INTRODUCTION
The idiopathic inflammatory myopathies traditionally comprise dermatomyositis, polymyositis, the anti-synthetase syndromes, immune-mediated necrotizing myopathy and inclusion body myositis. However, there are uncommon localized forms that are less known. In this review, we aimed to cover these uncommon forms.
AREAS COVERED
We identified rare forms of localized myositis on the basis of list provided by the homepage of the Neuromuscular disease center of Washington University, USA and on the basis of the authors' knowledge. We searched PubMed® for relevant articles on these forms with the aim of providing as much as possible information on their clinical manifestations as well as guidance on their work-up and treatment.
EXPERT OPINION
herein, we provide un updated description of rare forms of localized myositis. These forms are often difficult to diagnose because of their localized nature and are sometimes misdiagnosed as tumors. Knowledge about these rare forms of localized myositis can aid in their recognition and treatment.
Topics: Humans; Myositis; Polymyositis; Myositis, Inclusion Body; Autoimmune Diseases; Syndrome; Autoantibodies
PubMed: 36469645
DOI: 10.1080/1744666X.2023.2154655 -
Rheumatology (Oxford, England) Jul 2022
Topics: Adult; Cardiovascular Diseases; Dermatomyositis; Humans; Polymyositis
PubMed: 34962986
DOI: 10.1093/rheumatology/keab952