Anti-RNApol3-Associated myocarditis: an emerging disease linking autoimmunity and infection

• DOI: 10.1186/s13613-025-01443-1
• Publication Date: 2025-03-24T02:29:16Z
• Authors: Paul Quentric; Jean-Luc Charuel; Quentin Moyon; Guillaume Hékimian; Karim Dorgham; François Lifermann; Mathieu Kerneis; Alexis Mathian; Karim Aacha; Isabelle Melki; Juliette Chommeloux; Matthieu Petit; Melchior Gautier; Pierre Bay; Philippe Rouvier; Etienne Charpentier; Omaira da Mata-Ja...; Lucie Lefevre; Christophe Parizot; Ouriel Saura; David Levy; Sofia Ortuno; Matthieu Schmidt; Charles-Edouard Luyt; Guy Gorochov; Zahir Amoura; Alain Combes; Marc Pineton de C...
• Keywords: Fulminant; Pericardiocentesis

Abstract 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 systemic sclerosis, have been reported influenza-related FM. Our objective to provide additional insight into RNApol3-associated Methods We retrospectively included all admitted our institution between January 2013 and June 2023 acute positive serum RNApol3. compared their characteristics, etiologies, outcomes those of cohort RNApol3 negative myocarditis. Results Twenty-nine RNApol3-positive patients, comprising 83% females mean age 39 ± 12 years, were this study. Each patient was the intensive care unit at least once 11 (38%) relapsed. Triggers influenza virus 55% SARS-CoV-2 48% cases. The lowest left ventricular ejection fraction 10 [5-10] % highest troponin value 82 [22–360] times ULN. Patients required dobutamine (94%), veno-arterial extracorporeal membrane oxygenation (85%) pericardiocentesis (38%). At last follow-up, 76% still alive, while 7% had undergone cardiac transplantation, 3% assist device. Compared RNApol3-negative cases, associated female gender, fulminant evolution, tamponade, higher likelihood being caused proven viral infection, rate relapse. Conclusion an emerging disease linking autoimmunity infection unique cause acquired, pathogen-specific, organ-specific immunodeficiency. should be screened cases FM, especially young women infected RNA risk FM sclerosis needs further investigation.