ANCA阴性寡免疫复合物新月体肾炎患者的临床病理特征

Clinicopathological characteristics of antineutrophil cytoplasmic antibody negative and positive pauci-immune crescentic glomerulonephritis

目的比较抗中性粒细胞胞浆抗体(ANCA)阴性(ANCA-)与ANCA阳性(ANCA+)寡免疫复合物新月体肾炎(PICGN)临床病理特征及预后的异同。方法对住院肾活检确诊的75例PICGN患者进行流行病学、临床表现、病理特征及预后的回顾性分析。结果①ANCA-组发病年龄小于ANCA+组[(51.7±13.7)岁vs.(44.3±14.1)岁](P<0.05);②ANCA-组肉眼血尿发生率显著高于ANCA+组(56.7%vs.31.1%)(P<0.05),ANCA-组尿蛋白定量(3.7g/dvs.2.4g/d)和达到肾病范围蛋白尿患者比例(40.0%vs.15.6%)均显著高于ANCA+组(P<0.05),ANCA-组血红蛋白水平较高(9.2g/dlvs.7.8g/dl)(P<0.01);③病理检查:ANCA-组新月体形成比例显著低于ANCA+组(63.5%vs.77.8%)(P<0.01);④ANCA-组中位随访时间49个月(7～144个月),3例失访,无死亡病例。ANCA+组中位随访时间40月(6～120个月),3例失访,12例死亡。Kaplan-Meier图预测ANCA-组5年存活率显著高于ANCA+组(100.0%vs.58.0%)(P<0.01)。结论与ANCA+的PICGN相比,ANCA-患者尿检异常较重,然而肾活检新月体比例较低,慢性化病变较轻,肾外器官受累也较少,预后相对较好。

Objective To compare the clinicopathological characteristics and outcomes of the patients with antineutrophil cytoplasmic antibody （ANCA） positive and with ANCA negative pauciimmune crescentic glomerulonephritis（PICGN）. Methods Data of seventy-five Chinese patients with clinical and renal biopsy-proved PICGN were analyzed retrospectively. Results Compared the patients with ANCA-,the cases with ANCA＋ were older in age[（51.7±13.7） years old vs. （44.3 ±14.1） years old] （P〈0. 05）. Clinically, compared with the patients in ANCA- PICGN group, patients with positive ANCA had a lower level of urinary protein （2. 4 g/d vs. 3.7 g/d）（P〈0.05）, lower prevalence of nephrotic syndrome（15.6% vs. 40.0%）（P〈0. 05） and gross hematuria（31.1% vs. 56.7%）（P〈 0.05）. Pathologically, the degree of crescent formation was markedly higher in ANCA＋ than that in ANCA- PICGN patients（77.8% vs. 63. 5%）（P〈0. 01）. Death rate was 26.6% in ANCA＋ group while none in ANCA- group. The patients with ANCA＋ and ANCA- had been received a median follow-up of 40 （range 6- 120） and 49 （range 7- 144） months, respectively. The 5-year renal survival cumulative proportion of patients with ANCA＋ and ANCA- was 37.5% and 61.5% ,respectively. Conclusion The patients with ANCA＋ and ANCA- PICGN have many marked differences in clinical manifestations, renal histological lesion and prognosis. It may be the different characteristics that more nephrotic syndrome and gross hematuriathe, but less extrarenal involvement, milder renal insufficiency level, less chronic lesions, and better outcomes are more common in paitents with ANCA-- PICGN. ANCA-- PICGN might represent an independent disease entity from ANCA＋PICGN.