坏死性肉芽肿性血管炎临床诊治新进展——附96例临床分析  

Progress on clinical diagnosis and treatment of necrotizing granulomatous vasculitis： clinical analysis of 96 cases

作　　者：柴晶晶[1] 蔡柏蔷[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院呼吸内科,100730

出　　处：《国际呼吸杂志》2010年第14期835-840,共6页International Journal of Respiration

摘　　要：目的 回顾性分析坏死性肉芽肿性血管炎（necrotizing granulomatous vasculitis,NGV）的临床资料,提高对本病的认识.方法 对我院收治的96例NGV患者的临床资料进行回顾性分析.结果 男45例,女51例,发病年龄4～71岁,平均年龄（39±15）岁.在病程中,出现鼻部症状58.3%,耳部症状47.9%,眼部症状45.8%,下呼吸道症状60.4%,血尿、蛋白尿57.3%,贫血43.9%,但与肾功能不全无明显相关性.静脉血栓栓塞症4.2%,抗中性粒细胞胞浆抗体阳性率91.3%,其中蛋白酶3特异性抗中性粒细胞胞浆抗体79.3%,髓过氧化物酶特异性抗中性粒细胞胞浆抗体12.0%.胸部CT异常率92.1%,表现为肺部结节或肿块影76.3%,斑片状阴影38.2%,空洞形成21.1%.最常见的病理表现包括坏死、肉芽肿和（或）血管炎,但三者同时存在率低,不同的活检部位阳性率差别大.经治疗后患者的部分或完全缓解率为88.5%.结论 NGV的临床表现复杂多样,有多系统或器官病变的症状,以上、下呼吸道及肾脏受累最多见,病理特点主要为坏死性肉芽肿及小血管炎.治疗后缓解率高,但容易复发.Objective To improve the understanding of necrotizing granulomatous vasculitis （NGV） by retrospectively analyzing the clinical data of NGV. Methods The clinical data of 96 patients with NGV were retrospectively investigated. Results There were 45 males and 51 females. The onset age ranged from 4 to 71 years old. The mean age was （39±15） years old. In the course of disease, 58. 3% patients had nasal symptoms, 47. 9% patients had ear symptoms, 45. 8% patients had eye symptoms, 60. 4% patients had lower respiratory tract symptoms,57. 3% patients had hematuria,proteinuria,43. 9% patients had anemia, but there was not significant correlation with kidney disfunction. 4. 2% patients had venous thromboembolism. The positive rate of anti-neutrophil cytoplasmic antibody （ANCA） was 91. 3%, in which proteinase 3-ANCA was 79. 3 % , myeloperoxidase-ANCA was 12.0%. The abnormality rate of chest CT was 92.1%, in which pulmonary nodule or mass was 76.3%, patchy shadow was 38.2%, cavitation was 21.1%. The most common pathologic features were necrosis, granulomas and/or vasculitis, but the simultaneous rate of three features was low. The positive rates were different in different biopsy sites. After treatment, the partial or complete remission rate of patients was 88. 5%. Conclusions Clinical manifestations of NGV are complicated, multiple systems or organs are involved. The upper and lower respiratory tract and kidney are most commonly involved. The pathologic features are necrotizing granulomatosis and microvasculitis. After treatment,the remission rate was high, but the relapse was also common.

关 键 词：坏死性肉芽肿性血管炎 临床分析 

分 类 号：R593.2[医药卫生—内科学]