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Published on: June 20, 2014
Anti-RNApol3-Associated myocarditis: an emerging disease linking autoimmunity and infection
Paul Quentric1,2, Jean-Luc Charuel3, Quentin Moyon2,4
1Centre d'Immunologie et des Maladies Infectieuses (CIMI-Paris), Sorbonne Université, Inserm, Paris, France.
Background:
Fulminant myocarditis (FM) is a severe condition primarily triggered by viruses. Anti-RNA polymerase III autoantibodies (RNApol3) which are typically found in patients with severe systemic sclerosis, have been reported in patients with influenza-related FM. Our objective is to provide additional insight into RNApol3-associated FM.
Methods:
We retrospectively included all patients admitted to our institution between January 2013 and June 2023 with acute myocarditis and positive serum RNApol3. We compared their characteristics, etiologies, and outcomes with those of a cohort of RNApol3 negative acute myocarditis.
Results:
Twenty-nine RNApol3-positive patients, comprising 83% females with a mean age of 39 ± 12 years, were included in this study. Each patient was admitted to the intensive care unit at least once and 11 (38%) relapsed. Triggers included influenza virus in 55% and SARS-CoV-2 virus in 48% of cases. The lowest left ventricular ejection fraction was 10 [5-10] % and the highest troponin value was 82 [22-360] times the ULN. Patients required dobutamine (94%), veno-arterial extracorporeal membrane oxygenation (85%) and pericardiocentesis (38%). At the last follow-up, 76% of patients were still alive, while 7% had undergone cardiac transplantation, and 3% required a left ventricular assist device. Compared to RNApol3-negative cases, RNApol3-positive myocarditis was associated with female gender, fulminant evolution, tamponade, a higher likelihood of being caused by a proven viral infection, and a higher rate of relapse.
Conclusion:
RNApol3-associated myocarditis is an emerging disease linking autoimmunity and infection and a unique cause of acquired, pathogen-specific, organ-specific immunodeficiency. RNApol3 should be screened in all cases of FM, especially in young women infected by RNA viruses. The risk of FM in RNApol3-positive systemic sclerosis needs further investigation.
Insights
Anti-RNA polymerase III autoantibodies (RNApol3) are linked to severe myocarditis, particularly in young women. Screening for RNApol3 is crucial in fulminant myocarditis cases, especially those with viral infections, to identify this emerging autoimmune-infectious disease.
Area of Science:
- Cardiology
- Immunology
- Infectious Diseases
Background:
- Fulminant myocarditis (FM) is a severe, often viral, heart condition.
- Anti-RNA polymerase III autoantibodies (RNApol3), usually seen in systemic sclerosis, are linked to influenza-related FM.
Purpose of the Study:
- To investigate the characteristics, triggers, and outcomes of RNApol3-associated fulminant myocarditis.
- To compare RNApol3-positive FM with RNApol3-negative cases.
Main Methods:
- Retrospective analysis of patients with acute myocarditis and positive serum RNApol3 (2013-2023).
- Comparison of clinical data, etiologies, and outcomes against a cohort of RNApol3-negative acute myocarditis patients.
Main Results:
- Twenty-nine RNApol3-positive patients (83% female, mean age 39) experienced severe cardiac dysfunction (LVEF 10%, high troponin).
- Influenza (55%) and SARS-CoV-2 (48%) were common triggers; 38% relapsed. Intensive care and mechanical support (ECMO 85%) were frequently required.
- RNApol3-positive FM showed association with female gender, fulminant course, tamponade, viral etiology, and higher relapse rates compared to negative cases.
Conclusions:
- RNApol3-associated myocarditis represents a novel link between autoimmunity and infection, causing organ-specific immunodeficiency.
- Screening for RNApol3 is recommended in all FM cases, particularly in young women with RNA virus infections.
- Further research is needed on FM risk in RNApol3-positive systemic sclerosis patients.
