Cardiac Involvement in Eosinophilic Granulomatosis With Polyangiitis: A Retrospective Study in the Chinese Population.

Yingying Chen,Fengchun Zhang,Qingjun Wu,Hongxian Yang,Xiaoxiao Guo,Jing Li,Yan Zhao,Wen Zhang,Shangzhu Zhang,Jiaxin Zhou,Xiaofeng Zeng,Yunyun Fei

doi:10.3389/fmed.2020.583944

Abstract

Introduction: Cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) is associated with a poor prognosis and high mortality; however, few studies about cardiac involvement in EGPA in the Chinese population are available. We conducted this study to determine the clinical characteristics and overall outcomes of Chinese EGPA patients with cardiac involvement.Materials and Methods: We retrospectively collected the clinical data of 83 patients diagnosed with EGPA and analyzed the differences between the patients with and without cardiac involvement.Results: The prevalence of cardiac involvement in EGPA in this cohort was 27.7%. Compared with those without cardiac involvement, EGPA patients with cardiac involvement tended to have a younger age at onset (mean ± SD: 38.4 ± 10.5 vs. 42.1 ± 15.9 years, respectively, p = 0.039), higher eosinophil count (median [IQR]: 5810 [4020–11090] vs. 2880 [1530–6570] n/μL, respectively, p = 0.004), higher disease activity assessed using the Birmingham vasculitis activity score (BVAS) (median [IQR]: 20 [16–28] vs. 15 [12–18], respectively, p = 0.001), and poorer prognosis (Five Factor Score [FFS] ≥ 1: 100% vs. 38.3%, respectively, p = 0.001). In the cardiac involvement group, 43.5% of patients were asymptomatic, but cardiac abnormalities could be detected by cardiac examinations. With appropriate treatment, the overall outcomes of EGPA patients with cardiac involvement in our cohort were good, with only 3 (13.0%) patients dying in the acute phase and no patients dying during follow-up.Conclusions: Cardiac involvement in EGPA was associated with a younger age at onset, higher eosinophil count, higher disease activity, and a poorer prognosis. Comprehensive cardiac examinations and appropriate treatment are essential to improve the prognosis of those with cardiac involvement.

Highlights

Cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) is associated with a poor prognosis and high mortality; few studies about cardiac involvement in EGPA in the Chinese population are available
We retrospectively evaluated the data of 83 Chinese patients with EGPA to analyze and describe the prevalence, clinical characteristics, prognostic factors, and outcomes of EGPA patients with cardiac involvement to increase general understanding and provide meaningful information for physicians to use during clinical practice
The Birmingham vasculitis activity score (BVAS) [24] was used to assess disease activity and the revised Five Factor Score (FFS) [20] to evaluate the prognosis of all EGPA patients included in this study

Summary

Introduction

Cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA) is associated with a poor prognosis and high mortality; few studies about cardiac involvement in EGPA in the Chinese population are available. Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, was first described by Churg and Strauss as a rare autoimmune disease characterized by severe asthma, hypereosinophilia, and symptoms of necrotizing vasculitis in various organs [1]. It is classified as antineutrophil cytoplasmic antibody (ANCA)—associated systemic vasculitis affecting small- to medium-sized vessels [2], with ANCA positivity ranging from 26.2 to 85% [3,4,5,6,7,8,9]. We retrospectively evaluated the data of 83 Chinese patients with EGPA to analyze and describe the prevalence, clinical characteristics, prognostic factors, and outcomes of EGPA patients with cardiac involvement to increase general understanding and provide meaningful information for physicians to use during clinical practice

Methods

Results

Conclusion