儿童变应性肉芽肿性血管炎并发心内膜心肌病

Churg-Strauss syndrome in children: a case evolving in endomyocardiopathy

摘要:

患儿，女，13岁。因突眼6个月，发热伴咳嗽、活动后心悸、气促、乏力1个月入院。既往有哮喘、过敏性鼻炎等过敏性疾病史。体格检查：口唇紫绀，双肺闻及少量哮鸣音和细湿啰音，心音低钝，有奇脉。实验室检查：外周血嗜酸性粒细胞占64%(＞10%)，计数为6.7×109/L；骨髓嗜酸性粒细胞占31％，未见幼稚细胞。动脉血气分析为低氧血症。血IgE 2 472 IU/mL（显著升高）。抗核抗体（ANA）均质型阳性，其他自身抗体均阴性。X线示双肺野外带散在小斑片状影。超声心动图示右室心内膜增厚伴广泛附壁血栓形成，右室腔近于闭塞。临床诊断为儿童变应性肉芽肿性血管炎并发心内膜心肌病，给予足量泼尼松龙等治疗。患儿病情明显好转，血嗜酸性粒细胞计数、胸片均恢复正常，IgE降至1 678 IU/mL，但超声心动图无变化。2周后泼尼松龙逐渐减量，患儿接受心脏手术。病理结果为右室心内膜广泛附壁血栓形成，心内膜和心肌纤维化。术后小剂量泼尼松龙维持治疗，患儿康复后恢复上学。

Abstract:

A 13-year-old girl presented with a 1-month history of progressive exertional dyspnea (NYHA class Ⅳ) and exophthalmos for 6 months. She had a history of long-standing asthma and the presence of allergy. Hypereosinophilia and increased serum IgE levels (2 472 IU/mL) were observed. Chest radiography and a high resolution CT scan documented a massive interstitial pulmonary infiltration. Echocardiography confirmed mild tricuspid regurgitation, apical obliteration of the right ventricle by fibrocalcific thickening of the endocardium and echogenic material suggestive of thrombosis. Churg-Strauss syndrome with cardiac involvement (endomyocardiopathy) was diagnosed. The patient received anticoagulation and corticosteroid therapy. In view of rapidly progressive severe endomyocardiopathy and stable hematology, the patient was referred for cardiac surgery. Histopathological examination of resected specimens confirmed laminated thrombus but without any trace of eosinophils embedded.