目的：ANCA是指一组以中性粒细胞胞浆成分为抗原的自身抗体，与系统性血管炎关系密切，但亦有多篇文献报道ANCA还可见于SLE、 RA等结缔组织病，甚至在非结缔组织病，如溃疡性结肠炎也并不少见。本研究通过对ANCA阳性患者的疾病类型及临床特点进行病例回顾性分析，探讨ANCA对诊断和鉴别诊断风湿性疾病的临床意义。 方法：我院2005年1月至2008年9月期间，采用ELISA方法检测PR3-ANCA与MPO-ANCA的住院患者中，随机抽取ANCA阳性患者共104例。采用病例回顾性分析的方法，了解ANCA阳性患者的疾病谱，分别比较PR3-ANCA组与MPO-ANCA组、血管炎组与非血管炎组患者的临床表现及实验室检查结果的差异。采用SPSS11.5软件进行数据统计处理，包括描述性统计方法以及统计推断，统计推断采用χ²检验对分组的临床表现及实验室结果异常的频率进行统计学检验。 结果：一、104例ANCA阳性病例，血管炎患者22例(21.2%)，其中原发性ANCA相关性小血管炎(AAV)确诊13例。ANCA在本研究中对血管炎的阳性预测值仅为21.2%。二、非血管炎患者82例(78.8%)，中结缔组织病40例，包括SLE、RA；非结缔组织病42例，其中以溃疡性结肠炎(UC)最为常见。三、抗PR3抗体组与MPO-ANCA组对比，患者受累系统的均数分别为2.24及2.71， PR3-ANCA组胃肠道受累的发生率57.1%，高于MPO-ANCA组的27.1 %，差异有统计学意义(P<0.01)，而肾脏受累发生率24.5%低于后者的52.1%，差异有统计学意义(P<0.01)。四、血管炎组与非血管炎组对比，受累系统总数分别为3.55、2.28 (P<0.01)，其中血管炎组的上呼吸道、下呼吸道及肾脏受累频率分别为31.8%、50.0%和81.8%，均高于非血管炎组的3.7%、24.4%和26.8%，且差异均有统计学意义别为 P<0.01，P<0.05，P<0.05)；白细胞总数升高(≥10*10⁹/L)、血沉增快(≥50㎜)在血管炎组发生率为37.5%和63.0%，均明显高于非血管炎组，且差异有统计学意义(P<0.01，P<0.05)。五、ANCA阳性对血管炎的阳性预测值仅仅21.2%；当结合受累系统总数、临床表现及实验室检查可将ANCA对血管炎的阳性预测值由提高至24.4%-70%。 结论：ANCA阳性患者的疾病谱很广，并不局限于血管炎或结缔组织病，尤其在炎症性肠病中的阳性率较高。在综合型医院仅依靠ANCA阳性单一指标诊断血管炎疾病意义不大。结合临床特点及实验室检查综合分析，可以提高ANCA对于系统性血管炎的阳性预测值，从而提高ANCA在实际临床工作中的诊断意乂。 【关键词】抗中性粒细胞胞浆抗体(ANCA) PR3-ANCA MPO-ANCA 血管炎

 Objective To investigate the clinical significance of antineutrophil cytoplasmic antibodies(ANCA) in making diagnosis of vasculitis or other diseases. Methods From January 2005 to September 2008, 104 in-patients with ANCA positive detected by enzyme linked immunosorbent assay(ELISA) in People's Hospital of Peking University were selected. Clinical features and diagnoses were analyzed retrospectively. Result 1. Among 104 ANCA-positive patients, 22 were diagnosed vasculitis and 13 of them were ANCA associated vasculitis. 2.82 (78.8%) were non-vasculitis patients, and 40 of them were diagnosed connective diseases, such as SLEand RA. The rest 42 were non-connective patients, and Ulcerative colitis is the most common disease. 3. Further investigation of PR3-positive (n=49) and MPO-positive(n=48) revealed a greater extent of involved organs and more frequent involvement of the kidneys(P＜0.01) in MPO-positive, while less frequent involvement of gastrointestinal tract than in PR3-positive. 4. Compared to non-vasculitis, a greater extent of involved organs(2.28 vs 3.55, P＜0.01) and more frequent involvement of the upper/lower respiratory tract and the kidneys (P＜0.01,P＜0.05,P＜0.05, respectively)were found in the vasculitis patients. Elevated leucocyte and accelerated ESR (P＜0.01, P＜0.05, respectively) were also more common in vasculitis patients. 5. ANCA-positive combined with the extent of involved organs, clinical manifestations and laboratory findings, can increase the PPV to the diagnosis of vasculits. Results The disease spectrum of ANCA is quite extensive. To increase the diagnosis value, ANCA-positive should be combined with the clinical features. ANCA may also have clinical value in diagnosing other diseases. [ Key words ] Antineutrophil cytoplasmic antibodies, anti-PR3 antibody, anti-MPO antibody, vasculitis