IC14 (Atibuclimab) in Arrhythmogenic Cardiomyopathy

This proof-of-concept study will evaluate the safety, pharmacokinetics, and preliminary efficacy of IC14 administered via IV infusion in patients with ACM. In preclinical studies, anti-CD14 treatment prevented the development of ventricular dysfunction and cardiac damage in a mouse model of arrhythmogenic cardiomyopathy. The objective of this study is to determine whether IC14 treatment reduces markers of inflammation and disease biomarkers in ACM patients treated with IC14. Secondary objectives are to further characterize the effect of IC14 treatment on CCR2+ cell myocardial infiltration measured by myocardial positron emission tomography (PET)/CT imaging, ventricular premature contractions (VPCs), other arrhythmias, ICD discharges, NYHA functional classification, and quality of life. To characterize safety of IC14, the following assessments are to be performed: clinical biochemistry (safety analyses), ECG, ECHO, adverse events (AEs), serious adverse events (SAEs), and formation of anti-drug antibodies. Finally, pharmacokinetic/pharmacodynamic parameters will be conducted. These include blood test measurements levels of the drug and its binding to its target in the serum and on cells. This study will assign 5 patients to intravenous (IV) administration of IC14 (atibulcimab) at 20 mg/kg. The study drug will be administered every three weeks via IV infusion, for a total of 4 infusions (12 weeks of treatment).