Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors

Abstract

Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes. Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated via Kaplan–Meier curves. Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% versus 20%, p = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029–1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 109/L [area under the curve (AUC) = 0.692, p = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% versus 21.4%, p = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% versus 4.3%, p = 0.035) than the control group. Outcomes ( p = 0.131) and survival ( p = 0.1972) were not significantly different between the groups. Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.

Case Report: An Unusual Presentation of Cardiovascular Involvement in Eosinophilic Granulomatosis With Polyangiitis

“…Although EGPA belongs to the spectrum of ANCA-associated vasculitis, <50% of patients with EGPA are ANCA positive ( 1 ). Cardiac involvement occurs in approximately 15–60% of EGPA patients and can present as pericarditis, myocarditis, acute heart failure, acute myocardial infarction, valvular heart disease, and especially those who are ANCA negative ( 1 , 2 , 5 ). Löffler endocarditis is present in 50–60% hypereosinophilic syndrome (HES), but rarely reported in patient with EGPA ( 6 ).…”

“…Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis of small- and medium-size vessels, characterized by extravascular granulomas, eosinophilia, and tissue infiltration by eosinophils of multiple organs, including heart, lungs, skin, gastrointestinal tract, kidneys, and peripheral nerves ( 1 ). Cardiac involvement in EGPA is associated with a poor prognosis and high mortality.…”

“…Cardiac involvement in EGPA is associated with a poor prognosis and high mortality. Therefore, early diagnosis and treatment are essential to prevent the acceleration of cardiac involvement in patients with EGPA ( 1 , 2 ). We describe a rare case of EGPA characterized by involvement of heart and pulmonary artery.…”

Clinical Significance of MPO-ANCA in Eosinophilic Granulomatosis With Polyangiitis: Experience From a Longitudinal Chinese Cohort

“…Organ involvement was assessed on the basis of medical history, laboratory tests, imaging, and biopsies. Each system involvement was defined on the basis of our previous descriptions with slight modifications ( 11 ). Asthma was defined as the active condition that required continuous glucocorticoids (GC; including inhaled, oral, or intravenous form) or with persistent dyspnea.…”

Eosinophilic granulomatosis with polyangiitis: case report and literature review

“…ECG and echocardiography are recommended for all patients. Cardiac magnetic resonance imaging (MRI) is the most sensitive diagnostic technique and a valuable tool to detect cardiac involvement early ( figure 2c ) [ 29 , 30 ].…”