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目的分析变应性肉芽肿性血管炎(CSS)的临床特点,以提高诊治水平。方法回顾性分析1997年1月至2005年6月北京协和医院及天津市第一中心医院收治的26例CSS患者的临床资料(既往病史、临床表现、实验室检查、组织病理检查及治疗情况)。结果外周血嗜酸性粒细胞增多者占96·2%,哮喘发生率为84·6%,皮肤损害73·1%,肺浸润61·5%,多发单神经炎57·7%,消化系统受累46·2%,心血管系统损害42·3%,肾脏损害29·4%。病理检查血管外肉芽肿不多见。25例应用激素和免疫抑制剂治疗后好转。结论变应性肉芽肿性血管炎临床表现复杂,激素治疗反应好。有哮喘或嗜酸性粒细胞增多者需考虑CSS的可能,必要时行多系统多部位活检。
Objective To analyze the clinical features of allergic granulomatous vasculitis (CSS) in order to improve the diagnosis and treatment. Methods The clinical data (past medical history, clinical manifestations, laboratory tests, histopathological examination and treatment) of 26 CSS patients admitted to Peking Union Medical College Hospital and Tianjin No.1 Central Hospital from January 1997 to June 2005 were retrospectively analyzed. . Results Peripheral blood eosinophils accounted for 96.2%, asthma incidence 84.6%, skin lesions 73.1%, pulmonary infiltration 61.5%, multiple neuritis 57.7%, digestive system involvement 46.2%, cardiovascular system damage 42.3%, and kidney damage 29.4%. Pathological examination extravascular granuloma rare. 25 cases of application of hormones and immunosuppressive agents improved after treatment. Conclusions Allergic granulomatous vasculitis has complicated clinical manifestations and good response to hormone therapy. Patients with asthma or eosinophilia need to consider the possibility of CSS, if necessary, multi-system multi-site biopsy.