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Author Spotlight: Investigating the Pathophysiology of Eosinophilic Esophagitis
Published on: May 10, 2024
Cardiac involvement in eosinophilic granulomatosis with polyangiitis: acute eosinophilic myocarditis and chronic
Xiaohang Liu1, Yangzhong Zhou2, Jing Li2
1Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Insights
This study differentiates cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA), identifying eosinophilic myocarditis (EGPA-EM) as a severe subtype. A novel LATE-EAST score aids in early detection of high-risk EGPA-EM patients.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Cardiac involvement in EGPA is often broadly categorized, masking significant heterogeneity.
- Distinguishing between different cardiac manifestations is crucial for prognosis and treatment.
Purpose of the Study:
- To delineate the spectrum of cardiac involvement in EGPA.
- To identify distinct subtypes of cardiac disease in EGPA.
- To develop a tool for identifying high-risk populations within EGPA cardiac involvement.
Main Methods:
- Retrospective analysis of 193 EGPA patients (2012-2023) from a single center and public databases.
- Classification into eosinophilic myocarditis (EGPA-EM), inflammatory cardiomyopathy (EGPA-ICM), and control groups based on cardiac biomarkers, MRI, and biopsy.
- Comparison of clinical, laboratory, imaging, and prognostic data.
Main Results:
- EGPA-EM showed significantly higher eosinophil counts, lower ANCA positivity, and reduced lung involvement compared to EGPA-ICM and controls.
- EGPA-EM patients exhibited elevated cardiac troponin, reduced ejection fraction, and more ST-T abnormalities.
- EGPA-EM had a worse prognosis, with a 14.86% mortality rate and <50% 2-year event-free survival.
- The LATE-EAST score demonstrated high accuracy (AUC 0.85) in differentiating EGPA-EM from EGPA-ICM.
Conclusions:
- Cardiac involvement in EGPA comprises distinct subtypes, notably EGPA-EM, requiring specific attention.
- The LATE-EAST score is an effective tool for identifying high-risk EGPA-EM patients.
- Timely immunosuppressive therapy and investigation of targeted treatments like anti-IL-5 antibodies are warranted for EGPA-EM.
Objectives:
Currently, cardiac involvement is used to describe all eosinophilic granulomatosis with polyangiitis (EGPA) cardiac problems. However, heterogeneity exists among them. We aimed to depict the disease spectrum of EGPA cardiac involvement and identify the high-risk population.
Methods:
We included EGPA patients hospitalized in our centre from 2012 to 2023 and in public databases. Based on the cardiac enzymes, cardiac MRI and endomyocardial biopsy results, the patients were divided into three groups: eosinophilic myocarditis (EGPA-EM), chronic inflammatory cardiomyopathy (EGPA-ICM) and EGPA-Control. Their clinical, laboratory, imaging results and prognoses were collected and compared.
Results:
A total of 193 EGPA patients were included, 118 with cardiac involvement (74 EGPA-EM, 44 EGPA-ICM) and 75 control. Among EGPA-Control, EGPA-ICM and EGPA-EM, eosinophil increased (6.12/8.71/10.42 × 109/l, P < 0.01), ANCA positivity decreased (41.33/31.82/14.86%, P < 0.01) and lung involvement was reduced (73.33/72.73/43.24%, P = 0.02). In EGPA-EM, cardiac troponin was further elevated (0.27 vs 6.00 ng/ml, P < 0.01), ejection fractions decreased (57.79 vs 33.20%, P < 0.01) while more ST-T abnormality was observed (41.89 vs 20.45%, P = 0.02). The prognosis of EGPA-EM was significantly worse, with a 14.86% death rate and 2-year event-free survival rate below 50%. Furthermore, we proposed a LATE-EAST diagnostic score (7 items, 9 points) to discriminate EGPA-EM from EGPA-ICM using 4 points as threshold [area under the receiver operating characteristic curve 0.85 (95% CI 0.78-0.92), sensitivity 0.78, specificity 0.86].
Conclusions:
We first proposed different subtypes of cardiac involvement in EGPA. Identification and treatment of EGPA-EM needs improvement. LATE-EAST score could recognize the high-risk EGPA-EM effectively. Multi-disciplinary treatment is warranted, immunosuppressive therapy should be given in a timely manner and anti-IL-5 antibodies should be be tested in trials.
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