Churg-Strauss综合征并发急性冠状动脉综合征的荟萃分析

Acute Coronary Syndrome in Churg-Strauss Syndrome:3 Cases Report and Literature Review

目的分析Churg-Strauss综合征(CSS)并发急性冠状动脉综合征(ACS)患者的临床特点、治疗和结局。方法对北京协和医院就诊的3例及相关英文文献报道的8例CSS并发ACS患者的临床特点、冠状动脉造影(CAG)所见和治疗反应进行分析。结果本研究及文献报道的CSS并发ACS患者共11例,其中本研究3例、文献报道8例,男女比为4:7。11例ACS均出现在CSS发病初期,其中心肌梗死9例、心绞痛8例;CAG显示70%(7/10)为痉挛性多支血管狭窄或闭塞,动脉瘤1例。心脏外表现,包括支气管哮喘(11/11)、副鼻窦炎(7/11)、周围神经炎(5/11)、嗜酸性肺炎(4/11)及胃肠道受累(3/11)等。血清学特点为显著高嗜酸性粒细胞血症[(1.2～29.6)×109/L],抗中粒细胞胞浆抗体均阴性。CSS并发的ACS对常规冠心病二级预防治疗反应差,大剂量皮质激素和环磷酰胺联合治疗后病情稳定。结论 CSS并发的ACS以嗜酸性粒细胞相关的冠状动脉痉挛导致的多支血管严重狭窄或闭塞为特点,常规冠心病二级预防治疗反应差,联合应用大剂量皮质激素与环磷酰胺治疗可显著改善病情。

Objective To analyze the clinical features,therapy and prognosis of Churg-Strauss syndrome（CSS） complicated with acute coronary syndrome（ACS）.Methods Papers in English published between 1990 and 2010 in Medline database were searched.The reported myocardial infarction or pectoris angina complicating CSS were reviewed and 3 cases in our hospital were analyzed.Results Eleven cases with CSS complicated with ACS were reported,of which 4 patients were male,7 patients were female.All myocardial infarction or unstable angina pectoris occurred during early active phase of CSS.Acute pressuring chest pain was the most common and indicative feature of ACS,usually with corresponding findings in electrocardiogram and elevated cardiac enzymes.Angiography showed that multiple coronary vasospasm without anatomic stenosis were the main findings（7/10）,aneurysm was rare（1/10）.In addition,bronchial asthma（11/11）,parasinusitis（7/11）,peripheral neuropathy（5/11）,pulmonary infiltration（4/11） and gastrointestinal lesion（3/11） were presented.All cases presented with marked eosinophilia [（1.2-29.6）×109/L],but negative for anti-neutrophil cytoplasmic antibodies.Acute coronary syndrome might be refractory to vasodilator but sensitive to high-dose corticosteroid combined with cyclophosphamide.Conclusion ACS is a rare but life-threatening manifestation of CSS.Coronary spasm with eosinophilia might be the most important cause,which might be unresponsive to vasodilator.Management with high-dose corticosteroid and cyclophosphamide should be recommended,this might improve the prognosis.