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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 1 994年至 2 0 0 2年住院确诊抗GBM肾炎的 2 3例患者进行血清ANCA检测 ,对其中 1 1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 :2 3例抗GBM肾炎患者有 1 1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 0 9% ,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 (2 7 3 % ) ,C ANCA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 ,
Objective: To retrospectively analyze the clinical and pathological features of serum anti-neutrophil cytoplasmic antibody (ANCA) -positive anti-glomerular basement membrane (GBM) nephritis. Methods: Serum ANCA was detected in 23 cases of hospitalized diagnosed anti-GBM nephritis from 1949 to 2002 in the Institute of Nephrology of the People’s Liberation Army. Among them, 11 cases of ANCA positive patients were analyzed in terms of epidemiology, clinical and pathology And compared with serum ANCA-negative anti-GBM nephritis and ANCA-associated oligo-immune complex crescentic glomerulonephritis patients. Results: Serum ANCA was positive in 11 of 23 (47.8%) patients with GBM nephritis. Among them, 4 cases (36.4%) were positive for myeloperoxidase ANCA (MPO ANCA) and 1 case was positive for protease 3 ANCA (PR3 ANCA) [9 0 9%]. In this case, MPO ANCA and cytoplasmic ANCA C ANCA positive]), 3 (27.3%) were positive for perinuclear type (P ANCA) and 5 (45.5%) were positive for C ANCA. Serum ANCA-positive and -negative anti-GBM nephritis patients in the epidemiology, clinical manifestations, pathological changes and prognosis, etc. no difference, both with ANCA-associated oligo immune complex type crescentic glomerulonephritis patients compared to the age of onset, gender Ratio, oliguria (anuria) and the incidence of end-stage renal failure, crescentic nature, glomerulosclerosis, renal interstitial small vessel disease and prognosis were significantly different. Serum ANCA positive and negative anti-GBM nephritis patients response to treatment and prognosis are poor, ANCA-associated oligo immune complex type crescentic glomerulonephritis patients with relatively good prognosis. Conclusion: ANCA positive and negative serum anti-GBM crescentic nephritis may be a homologous disease,