Background/Purpose: To establish EGPA/Churg Strauss inflammatory heart disease prevalence and develop an algorithm for heart disease screening in EGPA patients.

Methods: An audit of all EGPA patients attending Addenbrooke’s was performed. Clinical presentation, cardiac studies, disease outcome measures and clinical course was noted. Values are given as percentages and median (IQR). Mann Whitney U was used.

Results: 131 EGPA patients (47% men) were followed of whom 96 (73%) underwent cardiac evaluation. Median age was 50 years (38 – 58), 37% were ANCA +ve and asthma preceded diagnosis in most by a median of 97 months (36 – 240). 41 of those screened (43%) were symptomatic for heart disease with: dyspnoea (47%), chest pain (29%), limb oedema (24%), palpitations (13%), syncope (4%), abdominal discomfort (2%) and shock (2%). 27/96 (28%) patients had EGPA-related heart disease in 20 of whom this was present at EGPA diagnosis, 5 developed it at time of EGPA flare and it preceeded EGPA diagnosis in two,. 59% (24) of those who were symptomatic and 5% (3) of those who did not have cardiac symptoms had EGPA heart disease. 15% patients had myocarditis, 6% pericarditis, 5% myopericarditis and 1 coronary vasospasm. One patient with pericarditis also had periaortitis. Cardiac abnormalities of any sort were found in 52% patients. Patients who had EGPA heart disease were younger (46 [28 – 52] V 50 [41 – 59]; p = 0.04), more frequently ANCA-negative (85% V 69%; NS), had higher BVAS scores (3 [1 – 4] V 1 [0.75 – 2]; p = 0.005), had higher eosinophil counts (5.60 [1.44 – 11,57] V 1.60 [0.75 – 4.00] x10⁹/L; p = 0.029) and higher CRP levels (52 [30 – 100] V 15 [5 – 81] mg/L; p = 0.017). Troponin I was determined in 33 patients and was elevated in 75% patients with EGPA inflammatory heart disease V 14% without (p = 0.001). Table 1 shows the percentage of cardiac abnormalities.

Conclusion: Twenty seven percent of EGPA patients have heart disease with 60% of those symptomatic for heart disease and 5% of those asymptomatic for it being affected. Our multiple comparisons suggest that EGPA patients with inflammatory heart disease have more aggressive systemic disease associated with higher markers of inflammation. All EGPA patients should have ECG, troponin and echocardiography as screening investigations with progression to cMRI for patients with heightened suspicion for cardiac disease. Table 1: Incidence of heart abnormalities in EGPA patients symptomatic and asymptomatic for heart disease. (n=100; 4 patients originally screened in the asymptomatic group later became symptomatic and were again tested)

BVAS = Birmingham Vasculitis Activity Score, ECG = Electrocardiogram, cMRI = Cardiac Magnetic Resonance Imaging, LGE = Late gadolinium enhancement, LVEF= Left ventricle ejective fraction, TTE = Transthoracic Echocardiography. p<0.05 was considered significant difference between those symptomatic and asymptomatic for heart disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/high-prevalence-of-inflammatory-heart-disease-in-eosinophillic-granulomatosis-with-polyangiitis-churg-strauss-patients/