Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by blood and tissue eosinophilia, and asthma. Its cardiac involvement is a major concern that was previously described as the most important predictor of death; however, definitions of that involvement differ according to study. We aimed to describe the first EGPA cardiac manifestations and outcomes in recent decades.

Methods: This retrospective, monocenter study included all EGPA patients followed in our vasculitis center. Patients’ and disease characteristics were recorded at diagnosis and throughout follow-up, especially cardiac assessments. Cardiac involvement was defined as clinical or extra-clinical signs of patent cardiopathy, with no other potential causes identified.EGPA relapses, major cardiac-related events (i.e. acute and chronic heart failure, atrial fibrillation and/or ventricular tachycardia, ischemic cardiopathy) and causes of death were recorded.

Results: The 150 EGPA patients (78 men; mean±SD age 47.8±15.9 years) included were characterized by asthma (99%), eosinophilia (95%), sinonasal abnormalities (84%), pulmonary infiltrates (51%), peripheral neuropathy (57%) and eosinophil-rich infiltrates (19%); 39% were ANCA+. Median baseline BVAS was 17.5 [IQR 13–22].

Sixty-five (43%) patients had cardiac involvement, 55 (85%) diagnosed at EGPA diagnosis and 10 (15%) during follow-up. The main clinical cardiac manifestations included chest pain (16%), peripheral edema (8%), palpitations (4%), cardiogenic shock (3%) and arrhythmia (1%). Patients with cardiac involvement, compared to those without, respectively, were less frequently ANCA+ (27% vs. 48%, P=0.02), had less frequent peripheral neuropathy (46% vs. 66%, P=0.02), and had higher eosinophil count (9748±7639 vs. 6346±5291/mm³, P=0.004).

Patients with cardiac involvement had abnormal ECG (70%), abnormal echocardiography [(68%; mainly pericardial effusion (42%) and left ventricular dysfunction (31%)] and cardiac MRI abnormalities [(98%; with left ventricular dysfunction (43%), myocardial edema (24%) and late gadolinium enhancement (74%)]. However, ECG, echocardiography and cardiac MRI, respectively, were also abnormal for 32%, 40% and 61% of the patients without patent cardiopathy.

After mean follow-up of 121±97.2 months, 65 (43%) patients experienced EGPA relapses, with no between-group differences (37 vs 48%, P=0.19). However, 46% of patients with cardiac involvement had cardiac relapses. Major heart-related events occurred in 17/150 (11%) patients, most often those with known cardiac involvement (18% vs. 6%, P=0.02). Finally, 4 patients died but none from cardiac causes.

Conclusion: Cardiac involvement is frequent in EGPA in a tertiary referral center, especially in patients without ANCA, with higher eosinophil counts and no peripheral neuropathy. Long-term outcome was better than previously reported. However, in the absence of a consensual definition of cardiac involvement, comparisons among studies remains difficult.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cardiac-involvement-of-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-initial-manifestations-and-outcomes-based-on-data-from-a-monocenter-patient-cohort/