ANCA相关性小血管炎51例临床分析

Analysis of the clinical manifestations in 51 patients with anti-neutrophil cytoplasmic autoantibodies-related vasculitis

目的: 回顾性分析抗中性粒细胞胞浆抗体(ANCA)相关性小血管炎(AASV)的临床特征、诊断、治疗及误诊原因,旨在提高对该病的认识。方法: 分析我院2003年1月至2011年1月确诊的51例AASV患者的临床资料。结果: 51例中60岁以上者占72.5%。cANCA阳性8例,均识别PR3;pAN-CA阳性41例,均识别MPO;2例ANCA阴性。20例行组织病理检查,12例有典型的血管炎表现。肾、肺受累率分别为94.1%和72.5%。80.4%患者首发表现为发热、乏力、纳差、消瘦等全身症状。全部病人血沉快、C反应蛋白高,多数病人有贫血、低蛋白血症、白细胞及血小板计数升高等。多数患者接受糖皮质激素和环磷酰胺治疗后病情明显改善。结论: AASV好发于老年男性。ANCA检测及组织病理可助早期诊断。糖皮质激素联合环磷酰胺疗效好。

Objective： To analyze the clinical features, diagnosis and treatment of anti - neutrophil cytoplasmic autoantibodies （ANCA） associated systemic vasculitis（AASV） in order to improve the understanding of this disorder. Methods： The clinical data of 51 patients with AASV diagnosed in our hospital from 2003 to 2011 were retrospec- tively analyzed. Results： Of the 51 patients, 30 were male and 21 female. Patients aged over 60 accounted for 72.5 %. cANCA was positive in 8 patients, in which all of them recognized proteinase 3 （PR3） ; pANCA was positivein 41 patients, in which all of them recognized myeloperoxidase（MPO） and only 2 were ANCA negative. The biopsies in 12 patients showed typical manifestation of small vessel vasculitis, such as tbcal segmental necrotizing vasculitis and necrotizing granulomatous inflammation. Kidney （94. 1% ） and lung （72.5 % ） were involved. Fever, fatigue, anorexia and weight loss were early symptoms, seen in 80.4% of the patients. In laboratory examination, all pa-tients had elevated ESR and CRP and most patients had anemia, hypoproteinemia, elevated WBC and PLT count. The rate of pre- admission inisdiagnosis was 88.2%. Most patients improved significantly ＇after king treated with glucocorticoid and cyclophosphamide. Conclusion： Patients with AASV were mainly old male. ANCA detection and pathology are helpful in the diagnosis. Treatment with glucocorticoid in combination with immunosuppressive agents is effective.