Churg—Strauss综合征的肺部病理形态学观察

Clinicopathologic study of Churg-Strauss syndrome

目的探讨Churg-Strauss综合征的临床及肺部病理特点。方法收集3例Churg-Strauss综合征患者的临床资料并进行分析，对3例肺组织（包括1例尸检和2例开胸肺活检标本）行4％甲醛固定，常规石蜡切片，HE染色，观察病理改变。结果2例男性，1例女性；年龄分别为68、58、12岁；3例均有反复哮喘，2例有外周血嗜酸性粒细胞计数升高，胸部CT示肺部有多发实变影，临床上有诊断Churg—Strauss综合征的线索。1例因累及心脏导致嗜酸性粒细胞浸润性心肌炎及血管炎，并发心肌梗死而死亡。显微镜下，3例均可见血管炎、血管周的过敏性肉芽肿、嗜酸性粒细胞肺炎和哮喘性支气管炎表现。结论应对Churg—Strauss综合征有充分的认识，对有相关症状的患者，应复查CT；一些病例临床体征虽不典型，但肺部有典型的病理改变，必要时需进行肺部活检。

Objective To study the clinical and pathologic features of Churg-Strauss syndrome （CCS）. Methods Three cases of Churg-Strauss syndrome, including 1 autopsy case and 2 cases with open thoracoscopic lung biopsy, were retrospectively reviewed. All the tissue samples were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. Results The first patient was a 68-year-old man who had history of asthma for 4 years, with recent exacerbation and chest pain for 2 weeks. Patient died 1 day after admission due to myocarditis and myocardial infarction. He did not have peripheral eosinophilia, skin or paranasal sinus pathology. CSS represented an incidental autopsy finding and he had never been treated with corticosteroid before. The other 2 patients were a 58-year-old male and a 12-year-old female, respectively. Both had history of asthma, peripheral eosinophilia and lung consolidations on computed tomographic examination. Pathologically, all cases showed vasculitis, perivascular allergic-type granulomas, eosinophilic pneumonia and asthmatic bronchitis. Conclusions Thorough understanding of the clinical and pathologic criteria is essential for arriving at a correct diagnosis of CSS. Although some patients may present with atypical symptoms, lung biopsies often reveal the classic histologic findings which include vasculitis and perivascular allergic granuloma formation.