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Heart disease in eosinophilic granulomatosis with polyangiitis (EGPA) patients: a screening approach proposal
Eloi Garcia-Vives1, J F Rodriguez-Palomares2, Len Harty3
1Autoimmune Systemic Diseases Unit, Internal Medicine Department, Hospital Universitari Vall d'Hebrón.
Insights
Prompt cardiac screening is crucial for eosinophilic granulomatosis and polyangiitis (EGPA) patients, as 45% of asymptomatic individuals showed cardiac abnormalities, enabling earlier diagnosis of heart disease.
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Eosinophilic granulomatosis and polyangiitis (EGPA) is a rare systemic vasculitis characterized by asthma, eosinophilia, and necrotizing vasculitis.
- Cardiac involvement is a significant cause of morbidity and mortality in EGPA patients.
- Current screening strategies for cardiac disease in EGPA are primarily symptom-driven, potentially leading to delayed diagnosis.
Purpose of the Study:
- To delineate the spectrum of cardiac manifestations in EGPA.
- To establish an evidence-based algorithm for cardiovascular evaluation in EGPA patients.
- To assess the utility of early cardiac screening in asymptomatic EGPA individuals.
Main Methods:
- Retrospective analysis of 131 EGPA patients from a specialized vasculitis clinic (1989-2016).
- Collection of clinical data and cardiovascular evaluation (CE) results, including troponin, ECG, echocardiography, and cardiac MRI.
- Comparison of CE findings based on symptom presence and diagnosis of inflammatory cardiac disease (ICD).
Main Results:
- Of 96 patients who underwent CE, 43% were symptomatic; 45% of asymptomatic patients had abnormal CE findings, leading to earlier diagnosis.
- 27 patients (21%) had EGPA-related ICD (EGPA-rICD), often presenting at diagnosis.
- EGPA-rICD patients were younger, more frequently ANCA-negative, and had higher BVAS, eosinophil counts, and CRP levels compared to those without ICD.
Conclusions:
- A significant proportion of asymptomatic EGPA patients exhibit cardiac abnormalities on baseline evaluation.
- Early diagnosis of cardiac disease in EGPA is facilitated by prompt cardiac screening.
- A universal cardiac screening approach is recommended for all EGPA patients, superseding symptom-based algorithms.
Objective:
To define the pattern of cardiac involvement in eosinophilic granulomatosis and polyangiitis (EGPA) and propose an algorithm for heart disease screening.
Methods:
This was a retrospective study of EGPA patients attending a specialized vasculitis clinic (1989-2016). Clinical characteristics and cardiovascular evaluation (CE) results (serum troponin, ECG, echocardiography and cardiac magnetic resonance) were collected and compared according to symptoms and inflammatory cardiac disease (ICD).
Results:
A total of 131 EGPA patients were included, of whom 96 (73%) had undergone CE. The median (interquartile range) age was 50 (38-58) years and 36% showed ANCA+. Asthma preceded diagnosis by a median of 97 (36-240) months. Among the 96 patients who underwent CE, 43% were symptomatic, with dyspnea (47%) and chest pain (29%) being the predominant symptoms. In asymptomatic patients, CE reported abnormalities in 45% of cases, with a subsequent earlier diagnosis (4 vs 11 months). Overall, 27 patients had EGPA-related ICD (EGPA-rICD) that was already present at diagnosis in 20 cases, preceded it in 2 cases and developed later in 5 cases. EGPA-rICD patients were younger (46 vs 50 years; P = 0.04), had more frequently abnormal ECG (30.8 vs 2.1%; P < 0.001), negative ANCA (85 vs 69%; NS), higher BVAS score (3 vs 1; P = 0.005), higher eosinophil count (5.60 vs 1.60 × 109/l; P = 0.029) and higher CRP (52 vs 15 mg/l; P = 0.017). Overall, 11% of cases with EGPA-rICD were asymptomatic.
Conclusion:
In our study, 45% of asymptomatic patients had an abnormal baseline cardiac evaluation, which allowed an earlier diagnosis of cardiac disease. We recommend prompt cardiac screening in all EGPA patients, instead of a symptoms-guided algorithm.
