血清抗中性粒细胞胞浆抗体阳性的抗肾小球基膜肾炎的临床与病理特征

目的：回顾性分析血清抗中性粒细胞胞浆抗体(ANCA)阳性抗肾小球基膜(GBM)肾炎的临床和病理特征。方法：对解放军肾脏病研究所1994年至2002年住院确诊抗GBM肾炎的23例患者进行血清ANCA检测，对其中11例ANCA阳性患者在流行病学、临床及病理等方面进行分析，并与血清ANCA阴性抗GBM肾炎及ANCA相关性寡免疫复合物型新月体肾炎患者进行比较。结果：23例抗GBM肾炎患者有11例(47．8％)同时存在血清ANCA阳性。其中髓过氧化物酶-ANCA(MPO-ANCA)阳性者4例(36．4％)，蛋白酶3-ANCA(PR3-ANCA)阳性者1例[9．09％，该例同时合并MPO-ANCA及胞浆型ANCA(C-ANCA阳性)]，核周型(P-ANCA)阳性者3例(27．3％)，DANCA阳性者5例(45．5％)。血清ANCA阳性和阴性抗GBM肾炎患者在流行病学、临床表现、病理改变及预后等方面无差异，二者与ANCA相关性寡免疫复合物型新月体肾炎患者相比，在发病年龄、性别比例、少尿(无尿)及就诊时终末期肾衰发生率、新月体性质、肾小球硬化、肾间质小血管病变及预后等方面差异显著。血清ANCA阳性和阴性抗GBM肾炎患者对治疗的反应和预后均很差，ANCA相关性寡免疫复合物型新月体肾炎患者预后相对较好。结论：血清ANCA阳性和阴性的抗GBM新月体肾炎可能为同源性疾病，而ANCA相关性寡免疫复合物型新月体肾炎与之不同。

Clinical and pathological features in anti-GBM crescentic glomerulonephritis patients with serum ANCAs

Objective:To investigate the epidemiological, clinical and pathological features of anti GBM crescentic glomerulonephritis(CGN)patients with coexisting serum anti neutrophil cytoplasmic antibodies (ANCA). Methodology:Twenty three sera from 23 anti GBM CGN patients were collected and were tested for ANCA respectively. The epidemiological, clinical and pathological features of patients with coexisting anti GBM antibody and ANCA were analyzed,and were made comparison of anti GBM CGN patients without coexisting ANCA and of ANCA associated pauci immune CGN patients. Results:Of the 23 sera with anti GBM antibody, 11 sera had coexisting ANCAs (4 MPO ANCA,1 PR3 ANCA incorporative with MPO ANCA and C ANCA , 3 P ANCA, 5 C ANCA),which represented 47 8% of the anti GBM CGN patients. No significant different epidemiological, clinical and pathological features between patients with and without coexisting serum ANCA. Both were different from ANCA associated pauci immune CGN patients in aspect of age, sex, ratio of olignuria/anuria, ESRD, character of crescents, glomerular sclerosis and vessel lesion, and had worse prognosis than the patients with ANCA associated pauci immune CGN. Conclusion:Anti GBM CGN patients with coexisting serum ANCA are similar to those anti GBM positive alone, but not similar to ANCA associated pauci immune CGN patients in epidemiological, clinical and pathological features as well as prognosis..