Mattia Zampieri1, Giacomo Emmi2, Matteo Beltrami1, Carlo Fumagalli1, Maria Letizia Urban3, Lorenzo-Lupo Dei1, Alberto Marchi1, Martina Berteotti1, Alessia Tomberli1, Katia Baldini1, Alessandra Bettiol3, Silvia Pradella4, Elena Silvestri3, Niccolò Marchionni5, Augusto Vaglio6, Iacopo Olivotto7, Domenico Prisco3. 1. Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy. 2. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. Electronic address: giacomo.emmi@unifi.it. 3. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy. 4. Radiology Unit, Careggi University Hospital, Florence, Italy. 5. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Department of Cardiothoracovascular Medicine, Careggi University Hospital, Florence, Italy. 6. Deptartment of Biomedical, Experimental and Clinical Sciences "Mario Serio", University of Firenze, Firenze, Italy. 7. Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy; Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Abstract
BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. Cardiac specific involvement (CSI) is caused by coronary artery vasculitis, but also by myocardial eosinophilic infiltration. To date, the prevalence of CSI associated with EGPA is unresolved. Aim of this study was to systematically assess the prevalence and clinical impact of CSI in a consecutive outpatient EGPA population. METHODS: Between October 2018 and July 2019, we prospectively enrolled 52 consecutive EGPA patients. All underwent comprehensive evaluation including a standardized questionnaire, physical examination, 12-lead-ECG, echocardiography. Cardiac magnetic resonance and 24 h-Holter were performed as deemed clinically appropriate. Cardiac abnormalities were defined as CSI based on the likelihood of their relation to EGPA vasculitis, after exclusion of alternative diagnoses. RESULTS: 52 enrolled patients, mean age 59±1 years. Thirteen of the 52 patients (25%) were classified as CSI+. CSI was characterized by myocarditis in four patients, non-scar-related regional wall motions abnormalities (RWMA) in three, apical thrombosis in two (one also had RWMA), pericarditis in three and non-atherosclerotic coronary disease (Prinzmetal angina and coronaritis) in 2. Five (38%) of the 13 CSI+ patients, presented an apical aneurysm. Peak eosinophil count at diagnosis was higher in CSI+: 8000 /μl vs CSI-: 3000 /μl, p = 0.017. Overall, 2 patients had severe LV dysfunction, 5 required urgent hospitalization and 8 required long-term cardioactive therapy. CONCLUSIONS: CSI was present in one-quarter of patients, often associated with high peak eosinophils. Myocarditis, RWMA and apical aneurysms were the most common manifestations. Although rarely severe and life-threatening, CSI often required long-term cardioactive treatment.
BACKGROUND:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. Cardiac specific involvement (CSI) is caused by coronary artery vasculitis, but also by myocardial eosinophilic infiltration. To date, the prevalence of CSI associated with EGPA is unresolved. Aim of this study was to systematically assess the prevalence and clinical impact of CSI in a consecutive outpatient EGPA population. METHODS: Between October 2018 and July 2019, we prospectively enrolled 52 consecutive EGPA patients. All underwent comprehensive evaluation including a standardized questionnaire, physical examination, 12-lead-ECG, echocardiography. Cardiac magnetic resonance and 24 h-Holter were performed as deemed clinically appropriate. Cardiac abnormalities were defined as CSI based on the likelihood of their relation to EGPA vasculitis, after exclusion of alternative diagnoses. RESULTS: 52 enrolled patients, mean age 59±1 years. Thirteen of the 52 patients (25%) were classified as CSI+. CSI was characterized by myocarditis in four patients, non-scar-related regional wall motions abnormalities (RWMA) in three, apical thrombosis in two (one also had RWMA), pericarditis in three and non-atherosclerotic coronary disease (Prinzmetal angina and coronaritis) in 2. Five (38%) of the 13 CSI+ patients, presented an apical aneurysm. Peak eosinophil count at diagnosis was higher in CSI+: 8000 /μl vs CSI-: 3000 /μl, p = 0.017. Overall, 2 patients had severe LV dysfunction, 5 required urgent hospitalization and 8 required long-term cardioactive therapy. CONCLUSIONS: CSI was present in one-quarter of patients, often associated with high peak eosinophils. Myocarditis, RWMA and apical aneurysms were the most common manifestations. Although rarely severe and life-threatening, CSI often required long-term cardioactive treatment.
Authors: Lilia M Sierra-Galan; Mona Bhatia; Angel Leovigildo Alberto-Delgado; Javier Madrazo-Shiordia; Carlos Salcido; Bernardo Santoyo; Eduardo Martinez; Maria Elena Soto Journal: Front Cardiovasc Med Date: 2022-07-13