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目的分析变应性肉芽肿性血管炎(Churg-Strauss综合征,CSS)的临床及病理学特点,提高诊断水平。方法复习确诊为CSS的4例患者的住院资料,对其临床表现及病理学特点进行总结。结果本病临床表现多种多样,以呼吸系统和周围神经受累最常见,所有患者末梢血的嗜酸性粒细胞比例增高(27.5%～73.3%)。3例肌肉活检显示血管炎性改变及嗜酸性粒细胞浸润;1例腓肠神经活检可见神经束内有髓纤维轴索变性,神经外膜血管炎性病变伴嗜酸粒细胞浸润。结论当患者出现呼吸系统和周围神经等多系统受累表现及末梢血嗜酸粒细胞比例增高时,应进行相关组织病理检查,及时诊断治疗。
Objective To analyze the clinical and pathological features of allergic granulomatous vasculitis (Churg-Strauss syndrome, CSS) and to improve the diagnostic level. Methods The hospitalization data of 4 patients diagnosed as CSS were reviewed, and their clinical manifestations and pathological features were summarized. Results The clinical manifestations of this disease varied, the respiratory system and peripheral nerve involvement are the most common, all patients with peripheral blood eosinophil increased (27.5% ~ 73.3%). 3 cases of muscle biopsy showed vascular inflammatory changes and eosinophil infiltration; 1 case of sural nerve biopsy showed myelinated nerve fiber bundle axonal degeneration, epicardial vascular inflammatory lesions with eosinophil infiltration. Conclusions When patients have multiple system involvement such as respiratory system and peripheral nerve, and the proportion of peripheral blood eosinophils increase, relevant histopathological examination should be carried out and timely diagnosis and treatment should be carried out.