136 A SHOCKING CASE OF MYOCARDITIS?

Abstract Background Cardiac involvement is common in systemic sclerosis (SSc), being a poor prognostic factor and the leading cause of mortality. ranges from serendipitous diagnosis to relevant cardiac manifestations such as arrhythmias, pericardial effusion, myocardial dysfunction, valvular diseases. Case report A 39-year-old female without remarkable medical history, on estroprogestinic therapy was admitted our hospital June 2020 after an out-of-hospital cardiovascular arrest due ventricular fibrillation (VF). coronary angiography ruled out artery disease. Magnetic resonance imaging (MRI) showed picture acute myopericarditis characterized by late linear nodular intramyocardial subepicardial gadolinium enhancement septal inferior segments left ventricle, with trivial oedema same location turbo inversion recovery magnitude (TIRM) sequences. subcutaneous-implantable defibrillator (S-ICD) positioned for secondary prevention. At 5 months ICD interrogation, 3 episodes VF detected treated device were documented. In December two more appropriate interventions polymorphic VT/VF occurred. biopsy then performed showing diffused replacement fibrosis absence signs inflammation or virus molecular investigation. March 2021 patient referred institution experiencing several arrhythmia successfully ICD. positron emission tomography (PET) scan documented uptake 18-fluorodeoxyglucose at middle basal septum. 'targeted' substitutive fibroadiposis sporadic inflammatory cells, cardiotropic viruses We initiated Anakinra, anti-IL1 agent anti-inflammatory effect prevent further development "inflammation-driven fibrosis". Given Raynaud's phenomenon reported patient, capillaroscopy performed, confirming isolated megacapillaries apical haemorrhages, compatible early pattern scleroderma. Immunologic examinations found homogeneous ANA 1:320 anti-RNA polymerase III, while other autoantibodies, viral serologies tubercular analysis negative. October 2021, readmitted recurrence arrhythmic storm. radiofrequency catheter-based ablation performed. After new episode VT properly S-ICD May 2022, explanted, resynchronization implanted. The genetic identified heterozygous deletion (c1262_1263del) Lamin (LMNA) classified probably pathogenic arrhythmogenic cardiomyopathy dilated cardiomyopathy. Conclusion borderline myocarditis anti-RNA-polymerase III+ LMNA finally formulated, having excluded pulmonary (PET, forced vital capacity, CO lung diffusion normal range), cutaneous, musculoskeletal gastro-oesophageal well paraneoplastic nature (by total body PET) given positivity antiRNA-polymerase III.