Chen Yingying, Guo Xiaoxiao, Zhou Jiaxin, Li Jing, Wu Qingjun, Yang Hongxian, Zhang Shangzhu, Fei Yunyun, Zhang Wen, Zhao Yan, Zhang Fengchun, Zeng Xiaofeng
Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Ministry of Health, Beijing, China.
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China.
Front Med (Lausanne). 2020 Dec 10;7:583944. doi: 10.3389/fmed.2020.583944. eCollection 2020.
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. We retrospectively collected the clinical data of 83 patients diagnosed with EGPA and analyzed the differences between the patients with and without cardiac involvement. 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, = 0.039), higher eosinophil count (median [IQR]: 5810 [4020-11090] vs. 2880 [1530-6570] n/μL, respectively, = 0.004), higher disease activity assessed using the Birmingham vasculitis activity score (BVAS) (median [IQR]: 20 [16-28] vs. 15 [12-18], respectively, = 0.001), and poorer prognosis (Five Factor Score [FFS] ≥ 1: 100% vs. 38.3%, respectively, = 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. 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.
嗜酸性肉芽肿性多血管炎(EGPA)累及心脏与预后不良和高死亡率相关;然而,针对中国人群EGPA心脏受累情况的研究较少。我们开展这项研究以确定中国EGPA心脏受累患者的临床特征和总体预后。我们回顾性收集了83例确诊为EGPA患者的临床资料,并分析了有、无心脏受累患者之间的差异。该队列中EGPA心脏受累的患病率为27.7%。与无心脏受累者相比,EGPA心脏受累患者发病年龄往往更小(平均±标准差:分别为38.4±10.5岁和42.1±15.9岁,P = 0.039),嗜酸性粒细胞计数更高(中位数[四分位间距]:分别为5810[4020 - 11090]和2880[1530 - 6570]个/μL,P = 0.004),使用伯明翰血管炎活动评分(BVAS)评估的疾病活动度更高(中位数[四分位间距]:分别为20[16 - 28]和15[12 - 18],P = 0.001),且预后更差(五因素评分[FFS]≥1:分别为100%和38.3%,P = 0.001)。在心脏受累组中,43.5%的患者无症状,但心脏检查可发现心脏异常。经过适当治疗,我们队列中EGPA心脏受累患者的总体预后良好,仅3例(13.0%)患者在急性期死亡,随访期间无患者死亡。EGPA心脏受累与发病年龄较小、嗜酸性粒细胞计数较高、疾病活动度较高及预后较差相关。全面的心脏检查和适当治疗对于改善心脏受累患者的预后至关重要。