Eosinophilic granulomatosis with polyangiitis, a new recurrent feature in an extremely rare disease

Abstract

Abstract Introduction Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare necrotizing vasculitis affecting small- and medium-sized vessels. EGPA may affect the heart leading to myocardial inflammation and necrosis. Although, only a few cardiological based studies have been conducted. Purpose This study aimed to investigate the prevalence and clinical impact of cardiac-specific involvement (CSI+) and to give an update on EGPA cardiological manifestations. Methods This is a single-centre study. Cardiological evaluation included ECG, blood test, echocardiography, global longitudinal strain (GLS), cardiac magnetic resonance (CMR). Results We prospectively enrolled 52 consecutive EGPA patients, between October 2018 and October 2019, mean age 59±3 years, 30 (57%) female. We identified 13 (25%) CSI+ patients: 6 myocarditis, 2 pericarditis, 1 coronaritis, 1 Prinzmetal angina, 2 LV apical thrombosis, 1 unexplained wall motions abnormalities (WMA) in the absence of coronary artery disease. Twelve-leads ECG revealed abnormalities in 11 (85%) CSI+ vs 9 (23%) CSI−, p=0.0001; ECG abnormalities identified CSI+ with 85% sensitivity, 77% specificity, 94% negative predictive value. Median troponin level in CSI+ 9 ng/L (IQR 6–11) vs CSI− 11 ng/L (IQR 6–25), p=0.2548; NT-pro-BNP value in CSI+ 210 pg/L (IQR 175–484) vs CSI− 159 ng/L (IQR 66–299), p=0.0576. Echocardiographic left ventricular end diastolic volume in CSI+ 62±4 ml/m2 vs CSI− 52±1 ml/m2, p=0.0116; LV ejection fraction in CSI+ 57%±2 vs CSI− 66%±1, p=0.0002. In CSI+ patients GLS was −15±1 vs CSI− GLS −21±0.4, p<0.0001. Echocardiography identified WMA in 8 (61%) CSI+ vs 1 (3%) CSI−. In 7 (54%) CSI+ patients, apical segments showed WMA and among them 5 demonstrated the presence of apical aneurysm. Twelve patients underwent CMR, it showed non ischaemic late gadolinium enhancement in 7 patients; in 5 patients we identified akinetic or dyskinetic segments without associated LGE. Two patients showed LV apical thrombus. Conclusions EGPA showed a high prevalence of CSI. We repeatedly found a tropism for apical involvement in WMA that often manifested themselves as apical aneurysm. These may be a new clinical feature, previously not described, in such a rare condition. Cardiac involvement EGPA Funding Acknowledgement Type of funding source: None