摘要
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.
出处
《中国麻风皮肤病杂志》
2011年第12期838-842,共5页
China Journal of Leprosy and Skin Diseases
关键词
抗体
抗中性白细胞胞质抗体
血管炎
antibodies
anfineutrophil cytoplasmic antibodies
vasculitis