坏死性肉芽肿性血管炎32例临床分析

目的分析坏死性肉芽肿性血管炎(NGV)的临床资料,加深对该病的认识。方法对新疆自治区人民医院2006年9月至2011年9月确诊的32例NGV患者的临床资料进行回顾性分析。结果 32例NGV患者中,男13例、女19例,发病年龄26⁶5岁,平均(32±15)岁。在病程中,出现鼻部症状为56.2%,下呼吸道症状为68.7%,血尿、蛋白尿为59.3%,贫血为46.7%。抗中性粒细胞胞质抗体(ANCA)阳性率为92.1%,其中蛋白酶3特异性ANCA为70.9%,髓过氧化物酶特异性ANCA为10.0%。胸部CT异常率为93.6%,表现为肺部结节或肿块影为75.3%,斑片状阴影为32.2%,空洞形成为19.3%。最常见的病理表现包括坏死、肉芽肿和/或血管炎。结论 NGV是一种多系统疾病,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。对可疑患者行ANCA血清学检查,影像学检查及组织活检有助于早期诊断,以改善预后。

Clinical Analysis of 32 Cases of Necrotizing Granulomatous Vasculitis

Objective To improve the understanding of necrotizing granulomatous vasculitis （NGV） by analyzing the clinical data of NGV. Methods The clinical data of 32 patients with NGV from People＇s Hospital of Xinjiang Uygur Autonomous Region during Sep. 2006 and Sep. 2011 were retrospectively investi- gated. Results There were 13 males and 19 females. The onset age ranged from 26 to 65 years old. The mean age was（32 ＋15）years old. In the course of disease,56.2% patients had nasal symptoms,68.7% patients had lower respiratory tract symtoms,59.3% patients had hematuria and proteinuria,46.7% patients had anemia. The positive rate of anti-neutrophil cytoplastic antibody （ANCA） was 92.1%, in which protein- ase 3-ANCA was 70.9% , myeloperoxidase-ANCA was 10. 0%. The abnormality rate of chest CT was 93.6% ,in which pulmonary nodule or mass was 75.3%, patchy shadow was 32.2% and cavitation was 19.3%. The most common pathologic features were necrosis, granulomas and/or vasculitis. Conclusion Clinical manifestations of NGV involve multiple systems and organs, among which the upper and lower respir- atory tract and kidney are mostly common. Suspected NGV patients should be given ANCA serology check, radiographic inspection and biopsy, which will help with the early diagnosis and improve the prognosis.