CARDIAC MANIFESTATIONS OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

Abstract

SESSION TITLE: Airways Function Measurement and Treatment OutcomesSESSION TYPE: Rapid Fire Original InvPRESENTED ON: 10/19/2022 11:15 am - 12:15 pmPURPOSE: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic disease characterized by asthma, peripheral & tissue eosinophilia & systemic vasculitis. Cardiovascular manifestations have been reported in prior studies, but the prevalence & spectrum of cardiac and vascular disease in EGPA is poorly understood.METHODS: The medical records were reviewed of a series of patients with EGPA who were evaluated from 1990-2021. For inclusion, patients had to fulfill standardized criteria for EGPA. Cardiac involvement was attributed to EGPA if findings could not be explained by other coexisting conditions.RESULTS: Two hundred and nineteen patients with EGPA were identified. The mean age at diagnosis was 52.16 years. 108 (49.3%) were female. Eighty-three (38%) had evidence of cardiac involvement. 49 (22.4%) had myocardial dysfunction. 6 (3%) with intra-cavitary cardiac thrombus; 22 (10%) with valvular involvement. 42 (19.2%) had pericardial disease (8 with tamponade and 1 with chronic constrictive pericarditis). Myocardial & pericardial involvement was also associated with greater degrees of peripheral eosinophilia -myocardial: 47, 18% vs no myocardial: 34, 20% of total WCC; p=0.0002 & pericardial: 47, 16% vs no pericardial 34, 20% of total WCC; p=0.0004. Patients with myocardial involvement with EGPA were less likely to be ANCA positive (24.5% versus 48.3%; p=0.002). ANCA positivity in those with pericardial involvement was 31.0% versus those without pericardial involvement 46.0%, p=0.077). Patients with myocardial involvement were less likely to have peripheral nerve involvement (a vasculitic manifestation) (52.1%) than those without cardiac involvement (77.7%; p=0.0008) but more likely to have pulmonary infiltrates/eosinophilic pneumonia (likely an eosinophilic manifestation) (70.8%) versus (49.4; p=0.0008). Cardiac involvement was associated with lower all-cause mortality over 10 years of follow-up (Hazard ratio 0.38 (0.15-0.98); p=0.03).CONCLUSIONS: In a large single-center series, both myocardial and pericardial involvement was common in EGPA and was associated with higher degrees of peripheral eosinophilia and the prevalence of eosinophilic pneumonia. A positive ANCA and peripheral nerve involvement were less common in those with myocardial and pericardial involvement.CLINICAL IMPLICATIONS: These findings may have implications for diagnosis, management including targeted treatment strategies of EGPA patients with cardiac involvement.DISCLOSURES: no disclosure on file for alvise berti;No relevant relationships by Garvan Kanepharmaceutical drug trial site invest. relationship with GSK Please note: $1-$1000 by Karina Keogh, value=Travelmonograph relationship with BMJ Please note: $1-$1000 by Karina Keogh, value=Honoraria SESSION TITLE: Airways Function Measurement and Treatment Outcomes SESSION TYPE: Rapid Fire Original Inv PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm PURPOSE: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic disease characterized by asthma, peripheral & tissue eosinophilia & systemic vasculitis. Cardiovascular manifestations have been reported in prior studies, but the prevalence & spectrum of cardiac and vascular disease in EGPA is poorly understood. METHODS: The medical records were reviewed of a series of patients with EGPA who were evaluated from 1990-2021. For inclusion, patients had to fulfill standardized criteria for EGPA. Cardiac involvement was attributed to EGPA if findings could not be explained by other coexisting conditions. RESULTS: Two hundred and nineteen patients with EGPA were identified. The mean age at diagnosis was 52.16 years. 108 (49.3%) were female. Eighty-three (38%) had evidence of cardiac involvement. 49 (22.4%) had myocardial dysfunction. 6 (3%) with intra-cavitary cardiac thrombus; 22 (10%) with valvular involvement. 42 (19.2%) had pericardial disease (8 with tamponade and 1 with chronic constrictive pericarditis). Myocardial & pericardial involvement was also associated with greater degrees of peripheral eosinophilia -myocardial: 47, 18% vs no myocardial: 34, 20% of total WCC; p=0.0002 & pericardial: 47, 16% vs no pericardial 34, 20% of total WCC; p=0.0004. Patients with myocardial involvement with EGPA were less likely to be ANCA positive (24.5% versus 48.3%; p=0.002). ANCA positivity in those with pericardial involvement was 31.0% versus those without pericardial involvement 46.0%, p=0.077). Patients with myocardial involvement were less likely to have peripheral nerve involvement (a vasculitic manifestation) (52.1%) than those without cardiac involvement (77.7%; p=0.0008) but more likely to have pulmonary infiltrates/eosinophilic pneumonia (likely an eosinophilic manifestation) (70.8%) versus (49.4; p=0.0008). Cardiac involvement was associated with lower all-cause mortality over 10 years of follow-up (Hazard ratio 0.38 (0.15-0.98); p=0.03). CONCLUSIONS: In a large single-center series, both myocardial and pericardial involvement was common in EGPA and was associated with higher degrees of peripheral eosinophilia and the prevalence of eosinophilic pneumonia. A positive ANCA and peripheral nerve involvement were less common in those with myocardial and pericardial involvement. CLINICAL IMPLICATIONS: These findings may have implications for diagnosis, management including targeted treatment strategies of EGPA patients with cardiac involvement. DISCLOSURES: no disclosure on file for alvise berti; No relevant relationships by Garvan Kane pharmaceutical drug trial site invest. relationship with GSK Please note: $1-$1000 by Karina Keogh, value=Travel monograph relationship with BMJ Please note: $1-$1000 by Karina Keogh, value=Honoraria