嗜酸性肉芽肿性多血管炎心脏受累16例临床分析

Clinical analysis of cardiac involvement in eosinophilic granulomatosis with polyangiitis:a report of 16 cases

目的分析嗜酸性肉芽肿性多血管炎(EGPA)心脏受累患者的临床特点,提高对EGPA心脏受累的认识。方法回顾性分析和总结山西白求恩医院2012年1月至2019年6月住院的16例EGPA心脏受累患者的临床资料。应用t检验、秩和检验、χ2检验进行分析。结果16例EGPA心脏受累,其中男性11例,女性5例;患者平均发病年龄(58±14)岁,与17例无心脏受累患者平均发病年龄比较(41±15)岁,差异有统计学意义(t=3.230,P<0.01),提示年龄大小与心脏受累与否有关。EGPA心脏受累16例患者中有症状者4例(25%),1例为首发症状,其余12例表现为心电图、心脏超声或心脏MRI异常,其中10例(62%)心电图异常,13例(81%)心脏超声检查异常,1例患者行心脏MRI提示心内膜炎。16例EGPA心脏受累患者中,肺脏受累14例,鼻受累10例,神经系统受累9例,皮肤受累9例,胃肠受累6例,肾脏受累2例。16例患者血嗜酸性粒细胞(EO)均增高,中位值2.46(1.49,3.94)×109/L,且EO与心脏受累有相关性。对核周型ANCA抗体阳性率分析,16例患者中仅2例患者阳性,与无心脏受累组(8例患者阳性)比较,差异有统计学意义(P=0.017)。16例患者均予糖皮质激素治疗,12例予免疫抑制剂治疗,其中10例用环磷酰胺。随访至今,1例因心力衰竭死亡,1例因脑出血死亡,3例失访,其余11例出院后均病情平稳。结论EGPA患者心脏受累发生率高,心脏各个结构均可以受累,多见于ANCA阴性患者,且心脏受累是预后不良因素之一。

Objective To analyze the clinical features of patients with cardic involvement in eosinophilic granulomatosis with polyangiitis(EGPA),and to enhance the understanding.Methods We retrospectively reviewed the clinical data of 16 patients with EGPA with cardiac involvement in Bethune hospital of Shan-xi from Jan 2012 to Jun 2019.T test,rank sum test andχ2 test were used for statistical analysis.Results 16 patients with cardiac involvement.There were 11 males and 9 female.The age of 16 patients with cardiac involvement ranged from 14 to 82 years old,and the average age of onset was(58±14)years.Compared with patients without cardiac invo-lvement was(41±15)years,the difference between the two groups was statistically significant(t=3.230,P<0.01).The analysis suggested that age was related to whether or not cardiac involvement.Cardiac related clinical symptoms occurred in 4 patients(25%).One patient presented with cardiac involvement as the initial symptom.The other 12 patients presented abnormal electrocardiogram(ECG),cardiac ultrasound or cardiac magnetic resonance imaging(MRI),including 10 patients(62%)with abnormal ECG,13 patients(81%)with abnormal cardiac ultrasound examination,and1patient with cardiac MRI suggesting endocarditis.Among 16 patients with EGPA cardiac involvement,14 presented with pulmonary involvement,10 cases with nasal involvement,9 cases with perip-heral neurological involvement,9 cases with skin involvement,6 cases with gastrointestinal involvement,2 cases with kidney damage.Eosinophils(EO)were increased in all 16 patients,with a median value of 2.46(1.49,3.94)×109/L,and EO was associated with cardiac involvement.Analysis of perinuclear anti-neutrophil cytoplasmic antibo-dies(pANCA)positive rate showed that only 2 of the 16 patients were positive.There was statistically significant difference(P=0.017)compared with the group without cardiac involvement(8 patients were positive).All 16 patients were treated with glucocorticoid,12 patients received immunosuppressive therapy,and 10 patients were treated with cyclophosphamide.During the ollow-up,1 case died of heart failure,1 case died of cerebral hemorrhage,3 cases were lost to follow-up,and the other 11 cases were all stable after discharge.Conclusion EGPA patients have a high incidence of cardiac involvement,and all cardiac stru-ctures can be involved,and most cardic involvement happens in ANCA negative patients.Cardiac involvement is one of the factors with poor prognosis.