100例新月体肾炎的免疫病理分型及临床病理分析

目的：了解新月体性肾炎的免疫病理分型及其临床病理特点。方法：对我科近10年来经肾活检确诊的100例新月体性肾炎进行回顾性分析，对患者血清进行抗中性粒细胞胞浆抗体（ANCA）和抗肾小球基底膜（GBM）抗体的检测。结果：100例患者中21％为抗GBM抗体型，其中6／21例同时合并ANCA阳性；47％为免疫复合物型，其中9／47型ANCA阳性；32％为少免疫沉积型，其中17／32例为ANCA阳性小血管炎。3种类型相比，抗GBM抗体型以青年男性为主，多有少尿或无尿（P＜0．05），肾小球受累受为广泛（P＜0．001），预后差（P＜0．001）。免疫复合物型以中青年为主，多表现为肾病综合征（P＜0．01），强化免疫抑制治疗有效。少免疫沉积型以中老年为主，有多系统受累（P＜0．05），治疗效果相对较好。结论：我国新月体肾炎中虽仍以免疫复合物型为主，但抗GBM抗体型和ANCA阳性小血管炎并不少见。肾活检免疫病理和血清自身抗体的联合应用对新月体肾炎进行分类更接近病因学诊断。

Immunopathological classification and clinicopathological analysis of 100 patients with crescentic glomerulonephritis

Objective To investigate the immunopathological classification and clinicopathological manifestations of patients with crescentic glomerulonephritis. Methods 100 patients with renal biopsy proven crescentic glomerulonephritis were retrospectively studied, whose serum ANCA and anti-GBM antibody were measured by ELISA. Results 21% of the patients were anti-GBM antibody type and 6/21 cases were also ANCA positive; 47% were immune complex type and 9/47 cases were ANCA positive; 32% were pauci-immune type and 17/32 cases were ANCA positive vasculitis. Patients with anti-GBM antibody were mainly young male with the worse prognosis( P <0.001), and majority of them had oliguria or suns clinically (P <0. 05) and extensive glomerular crescents formation pathologically (P <0. 001) . Patients with immune complex type were mainly young or middle aged, and majority of them had nephrotic syndrome (P <0. 01), meanwhile intensive immunospuppressive therapy was effective. Patients with pauci-immune type were mainly middle aged or elderly with multi-system involvement( P <0. 05), and had a relatively better response to intensive immunosuppressive therapy. Conclusions Although immune complex type is still in majority, anti-GBM antibody and ANCA related crescentic glomerulonephritis are not less common in China. Serum autoantibody markers combined with immunopathological classification are closer to etiology classification.