嗜酸粒细胞增多肌痛综合征临床分析

目的 分析嗜酸粒细胞增多肌痛综合征(EMS)的临床特点.方法 对北京协和医院9年来诊断为嗜酸粒细胞增多症的89例患者进行回顾性分析和随访,其中符合美国疾病控制中心(CDC)1996年EMS诊断标准的患者有4例,对其临床表现、治疗反应和预后进行全面分析.结果 4例EMS患者均除外感染、肿瘤等其他可以引起嗜酸粒细胞升高的疾病.均为女性,平均年龄48.8岁.血嗜酸粒细胞分类平均为0.4275(0.3120～0.7000),肌痛为主要表现,皮肤、关节、肺、神经病变等多系统累及较为突出.急性期对糖皮质激素反应敏感,一旦出现重度肺动脉高压则预后差.本病与环境因素关系密切,应注意患者的特殊用药史或饮食史.结论 EMS是一种多系统受累、以嗜酸粒细胞增多和肌痛为特征的综合征,急性期对糖皮质激素的应用很重要.

Clinical analysis of patients with eosinophilia-myalgia syndrome

Objective To analyze clinical features of patients with eosinophilia-myalgia syndrome (EMS).Methods Clinical manifestations,response to treatment and prognosis of 89 cases with eosinophilia hospitalized at Peking Union Medical College Hospital(PUMCH)during the past nine years were analyzed retrospectively,including four cases conformed with the diagnostic criteria for EMS set by the Centers for Disease Control and Prevention of the US.Results Infection,tumor and other diseases that could cause increased eosinophil count were excluded in all but four cases,with all female,average age of 48.8 years,and mean count of eosinophil of 42.75 percent of blood cells.Multi-organs and systems were involved in the patients,with the most common clinical manifestations of myalgia,skin rash,joint swelling,arthralgias,respiratory symptoms and paresthesia.Patients responded well to prednisone at acute phase of illness,but prognosis was poor as severe pulmonary hypertension occurred.Occurrence of EMS closely associated with environmental factors and special attention should be paid to patient's history of medication and diet in its diagnosis.Conclusion EMS is characterized by the increasing of eosinophils and myalgia,while multi-organs and systems were involved.Glucocorticoid plays an important role in the treatment at acute phase of illness.