ArticleEuropean heart journal2018
An autoantibody identifies arrhythmogenic right ventricular cardiomyopathy and participates in its pathogenesis.
Article in European heart journal, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 77 papers, 1 of them a synthesis that pooled it.
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Who cites it
77 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease.Biomolecules · 2022Pooled it
- Yield of Family Screening in Arrhythmogenic Right Ventricular Cardiomyopathy Without a Validated Genetic Cause.Circulation. Arrhythmia and electrophysiology · 2026Article
- From inflammation to inheritance: rethinking myocarditis as the first signal of desmosomal cardiomyopathy.The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of Cardiology · 2026Review
- Acute and Chronic Myocarditis in Men and Women.Current heart failure reports · 2026Review
- Integrative Molecular Analyses of Inflammatory and Autoimmune Signals in Cardiac Sarcoidosis.Circulation · 2026Article
- Clinical value of anti-desmoglein-2 antibodies in arrhythmogenic right ventricular cardiomyopathy: real-world evidence.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2026Article
- Review
- Autoantibody Profiling in Cardiomyopathies: Toward Immune-Guided Risk Stratification and Therapy.Journal of clinical medicine · 2026Review
- Apremilast improves cardiomyocyte cohesion and arrhythmia in different models for arrhythmogenic cardiomyopathy.Stem cell research & therapy · 2025Article
- Arrhythmogenic Cardiomyopathy and Biomarkers: A Promising Perspective?Journal of clinical medicine · 2025Review
- Cardiology's best friend: Using naturally occurring disease in dogs to understand heart disease in humans.Journal of molecular and cellular cardiology plus · 2025Review
- Late-phase depolarization and repolarization abnormalities in english bulldogs with phenotypic expression of arrhythmogenic cardiomyopathy.Veterinary research communications · 2025Article
- Intercalated Disc Abnormalities Are Linked to Arrhythmias in Inflammatory Cardiomyopathy.JACC. Clinical electrophysiology · 2025Article
- Cardiac Magnetic Resonance Guidance for the Pathogenetic Definition of Cardiomyopathies.Current cardiology reports · 2025Review
- Rapid progression of right ventricular dysfunction: a case report.BMC cardiovascular disorders · 2025Article
- Analysis of effector/memory regulatory T cells from arrhythmogenic cardiomyopathy patients identified IL-32 as a novel player in ACM pathogenesis.Cell death & disease · 2025Article
- Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies.Current cardiology reports · 2025Review
- Hot Phases Cardiomyopathy: Pathophysiology, Diagnostic Challenges, and Emerging Therapies.Current cardiology reports · 2025Review
- Review
- Prevalence and Correlates of Anti-DSG2 Antibodies in Arrhythmogenic Right Ventricular Cardiomyopathy and Myocarditis: Immunological Insights from a Multicenter Study.Journal of clinical medicine · 2024Article
17 more citing papers are in PubMed but not listed here.
Corrections and comments
- Commented on by
- Erratum issued
Authors and funding
19 authors.
Funding
Abstract
Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by right ventricular myocardial replacement and life-threatening ventricular arrhythmias. Desmosomal gene mutations are sometimes identified, but clinical and genetic diagnosis remains challenging. Desmosomal skin disorders can be caused by desmosomal gene mutations or autoantibodies. We sought to determine if anti-desmosome antibodies are present in subjects with ARVC. Methods and results: We evaluated ARVC subjects and controls for antibodies to cardiac desmosomal cadherin proteins. Desmoglein-2 (DSG2), desmocollin-2, and N-cadherin proteins on western blots were exposed to sera, in primary and validation cohorts of subjects and controls, as well as the naturally occurring Boxer dog model of ARVC. We identified anti-DSG2 antibodies in 12/12 and 25/25 definite ARVC cohorts and 7/8 borderline subjects. Antibody was absent in 11/12, faint in 1/12, and absent in 20/20 of two control cohorts. Anti-DSG2 antibodies were present in 10/10 Boxer dogs with ARVC, and absent in 18/18 without. In humans, the level of anti-DSG2 antibodies correlated with the burden of premature ventricular contractions (r = 0.70), and antibodies caused gap junction dysfunction, a common feature of ARVC, in vitro. Anti-DSG2 antibodies were present in ARVC subjects regardless of whether an underlying mutation was identified, or which mutation was present. A disease-specific DSG2 epitope was identified. Conclusion: Anti-DSG2 antibodies are a sensitive and specific biomarker for ARVC. The development of autoimmunity as a result of target-related mutations is unique. Anti-DSG2 antibodies likely explain the cardiac inflammation that is frequently identified in ARVC and may represent a new therapeutic target.
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