特发性嗜酸性粒细胞增多综合征的特征是嗜酸性粒细胞过度产生并伴有组织浸润,导致多器官功能障碍。其异质性表现使诊断具有挑战性且容易错过。一名70多岁的妇女因胸痛和呼吸急促而入院。诊断测试显示心肌酶升高,45%的射血分数和心包积液。心包穿刺术对她的症状有显著帮助。心导管插入术显示冠状动脉未闭。她被诊断患有心肌心包炎,并使用非甾体抗炎药出院。第二周她因胸痛加重而回来,呼吸困难和腹泻。胸部成像显示双侧浸润。诊断测试显示外周血中嗜酸性粒细胞占优势(59%),心包液(37%)和支气管肺泡灌洗(31%)。经过阴性感染检查,她开始使用糖皮质激素,反应良好。她服用类固醇后出院。美泊利单抗是门诊开始的,停用类固醇.在监测她的症状和嗜酸性粒细胞计数的同时,美泊利单抗在2年后停用。
Idiopathic hypereosinophilic syndrome is characterised by the overproduction of eosinophils with tissue infiltration, leading to multiorgan dysfunction. Its heterogenous presentation makes the diagnosis challenging and easy to miss. A woman in her 70s was admitted with chest pain and shortness of breath. Diagnostic testing showed elevated cardiac enzymes, an ejection fraction of 45% and pericardial effusion. Pericardiocentesis helped her symptoms significantly. Cardiac catheterisation revealed patent coronary arteries. She was diagnosed with myopericarditis and discharged on non-steroidal anti-inflammatory drugs. She returned the following week with worsening chest pain, dyspnoea and diarrhoea. Chest imaging showed bilateral infiltrates. Diagnostic testing showed eosinophilic predominance in peripheral blood (59%), pericardial fluid (37%) and bronchoalveolar lavage (31%). After a negative infectious workup, she was started on glucocorticoids and responded favourably. She was discharged on steroids. Mepolizumab was initiated outpatient, and steroids were discontinued. Mepolizumab was discontinued after 2 years while monitoring her symptoms and eosinophil counts.