Idiopathic inflammatory myopathies

Lundberg, Ingrid E.; Fujimoto, Manabu; Vencovský, Jiří; Aggarwal, Rohit; Holmqvist, Marie; Christopher‐Stine, Lisa; Mammen, Andrew L.; Miller, Frederick W.

doi:10.1038/s41572-021-00321-x

Cited by 347 publications

(310 citation statements)

References 245 publications

Supporting

Mentioning

276

Contrasting

Unclassified

Order By: Relevance

“…Idiopathic inflammatory myopathies (IIMs), also referred to generally as myositis, are a group of rare, chronic inflammatory autoimmune disorders, with the annual incidence and prevalence rate of 5.8-7.9 per 100,000 person-years, 14.0-17.4 per 100,000 person-years, respectively (1). IIMs mainly include dermatomyositis (DM), polymyositis (PM), inclusion body myositis, autoimmune necrotizing myopathy, overlap myositis and antisynthetase syndrome (2). DM and PM are the most common subtypes, which are characterized by inflammatory infiltration of the skeletal muscle and progressive proximal muscle weakness.…”

Section: Introductionmentioning

confidence: 99%

Coronary Heart Disease and Cardiovascular Risk Factors in Patients With Idiopathic Inflammatory Myopathies: A Systemic Review and Meta-Analysis

Luo

Chen

et al. 2022

Front. Med.

View full text Add to dashboard Cite

Objectives:It is well-established that the association between atherosclerotic cardiovascular diseases (ASCVD) and connective tissue diseases (CTDs), but the relationship between coronary heart disease (CHD) and idiopathic inflammatory myopathies (IIMs) remains controversial yet. The aim of this meta-analysis is to systematically evaluate the risk of CHD in IIMs patients. In addition, we explore differences in traditional cardiovascular risk factors between IIMs patients and controls.Methods:We searched Pubmed, EMBASE and Cochrane databases to identify relevant observational studies published in English up to August 2021. Pooled relative risk (RR) and 95% confidence interval (CI) was calculated using the generic inverse variance method for the risk of CHD. A meta-proportion analysis was conducted to assess differences in cardiovascular risk factors between two groups.Results:A total of 15 studies met inclusion criteria: seven studies focused on CHD and nine studies focused on traditional cardiovascular risk factors. The results demonstrated that IIMs patients had a higher risk of CHD (RR = 2.19, 95% CI: 1.40–3.42). Hypertension (OR = 1.44, 95% CI: 1.28–1.61), diabetes mellitus (OR = 1.67, 95% CI: 1.55–1.81) and dyslipidemia (OR = 1.48, 95% CI: 1.19–1.84) were more prevalent in IIMs patients compared with controls. However, there was a significant heterogeneity among studies assessing the risk of CHD and hypertension. Subgroup analysis demonstrated that definition of CHD, country and sample size may be potential sources of heterogeneity.Conclusions:IIMs patients were at increased risk of CHD, and traditional cardiovascular risk factors appeared more prevalent in IIMs patients. This systemic review offers the proof that early appropriate interventions could reduce cardiovascular-associated morbidity and mortality in IIMs patients.

show abstract

Section: Introductionmentioning

confidence: 99%

Coronary Heart Disease and Cardiovascular Risk Factors in Patients With Idiopathic Inflammatory Myopathies: A Systemic Review and Meta-Analysis

Luo

Chen

et al. 2022

Front. Med.

View full text Add to dashboard Cite

show abstract

“…A currently accepted classification divides myositis into clinicalserological categories: dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathies, polymyositis, and inclusion-body myopathy. Concerning muscle pathology, dermatomyositis is characterized by B and CD4 T cell infiltrate with perivascular distribution and complement activation, whereas in polymyositis inflammation appears at endomysial level, with a mononuclear cell infiltrate mainly composed by CD8 T cells and macrophages (7,(9)(10)(11). However, since each category might include heterogenous entities, a classification system based on myositis-specific antibodies has been advocated (6).…”

Section: Introductionmentioning

confidence: 99%

Anti-MDA5 Antibody Linking COVID-19, Type I Interferon, and Autoimmunity: A Case Report and Systematic Literature Review

et al. 2022

View full text Add to dashboard Cite

IntroductionThe SARS-CoV-2 infection has been advocated as an environmental trigger for autoimmune diseases, and a paradigmatic example comes from similarities between COVID-19 and the myositis-spectrum disease associated with antibodies against the melanoma differentiation antigen 5 (MDA5) in terms of clinical features, lung involvement, and immune mechanisms, particularly type I interferons (IFN).Case ReportWe report a case of anti-MDA5 syndrome with skin manifestations, constitutional symptoms, and cardiomyopathy following a proven SARS-CoV-2 infection.Systematic Literature ReviewWe systematically searched for publications on inflammatory myositis associated with COVID-19. We describe the main clinical, immunological, and demographic features, focusing our attention on the anti-MDA5 syndrome.DiscussionMDA5 is a pattern recognition receptor essential in the immune response against viruses and this may contribute to explain the production of anti-MDA5 antibodies in some SARS-CoV-2 infected patients. The activation of MDA5 induces the synthesis of type I IFN with an antiviral role, inversely correlated with COVID-19 severity. Conversely, elevated type I IFN levels correlate with disease activity in anti-MDA5 syndrome. While recognizing this ia broad area of uncertainty, we speculate that the strong type I IFN response observed in patients with anti-MDA5 syndrome, might harbor protective effects against viral infections, including COVID-19.

show abstract

“…Idiopathic inflammatory myopathies often affect extramuscular organs such as the skin, joints, lungs, heart and gastrointestinal tract ( 1 ). In particular, interstitial lung disease (ILD) is one of the major manifestations associated with poor mortality in patients with polymyositis (PM)/dermatomyositis (DM) ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

Mortality Risk Stratification Using Cluster Analysis in Patients With Myositis-Associated Interstitial Lung Disease Receiving Initial Triple-Combination Therapy

et al. 2022

View full text Add to dashboard Cite

ObjectiveTo stratify patients with polymyositis/dermatomyositis-associated interstitial lung disease (ILD) who were initially treated with an intensive regimen consisting of high-dose corticosteroids, a calcineurin inhibitor, and intravenous cyclophosphamide (triple-combo therapy) into subgroups based on mortality outcomes by a cluster analysis using a large-scale multicenter retrospective cohort of Japanese patients with myositis-associated ILD (JAMI).MethodsTwo-step cluster analysis of preclustering and subsequent hierarchical clustering was conducted in 185 patients who received triple-combo therapy in an unbiased manner. Initial predictors for mortality previously reported in patients with myositis-associated ILD were used as variables and included age, sex, disease duration, classification of myositis, requirement of supplemental oxygen, anti-aminoacyl tRNA synthetase (ARS) antibody, anti-melanoma differentiation-associated gene 5 (MDA5) antibody, and serum levels of C-reactive protein (CRP) and Krebs von den Lungen-6 (KL-6). The cluster model was further applied to 283 patients who received conventional regimens consisting of corticosteroids with or without a single immunosuppressive agent (dual-combo therapy or monotherapy). Cumulative survival rates were compared using Kaplan-Meier analysis, and the log-rank test was used to test for significant differences between two groups.ResultsWe developed a cluster model consisting of 6 clusters, which were categorized by age at onset, clinically amyopathic dermatomyositis, CRP, KL-6, requirement of supplemental oxygen, anti-ARS antibody, and anti-MDA5 antibody. This model was judged to be of good quality based on the silhouette measure of cohesion and separation of 0.6. These clusters were regrouped into three subsets based on low (<10%), moderate (10-50%), and high (>50%) mortality rates. The performance of the clustering was generally replicated in patients who received initial dual-combo therapy or monotherapy. Survival benefits of triple-combo therapy over dual-combo therapy or monotherapy were not observed in any of the clusters.ConclusionWe successfully developed a cluster model that stratified patients with myositis-associated ILD who were treated with initial triple-combo therapy into subgroups with different prognoses, although this model failed to identify a patient subgroup that showed survival benefits from triple-combo therapy over dual-combo therapy or monotherapy.

show abstract

Idiopathic inflammatory myopathies

Cited by 347 publications

References 245 publications

Coronary Heart Disease and Cardiovascular Risk Factors in Patients With Idiopathic Inflammatory Myopathies: A Systemic Review and Meta-Analysis

Coronary Heart Disease and Cardiovascular Risk Factors in Patients With Idiopathic Inflammatory Myopathies: A Systemic Review and Meta-Analysis

Anti-MDA5 Antibody Linking COVID-19, Type I Interferon, and Autoimmunity: A Case Report and Systematic Literature Review

Mortality Risk Stratification Using Cluster Analysis in Patients With Myositis-Associated Interstitial Lung Disease Receiving Initial Triple-Combination Therapy

Contact Info

Product

Resources

About