抗肾小球基膜病合并IgA肾病或膜性肾病的临床病理特征分析

Anti-glomerular basement membrane disease combined with IgA nephropathy or membranous nephropathy

目的:探讨抗肾小球基膜(GBM)病合并IgA肾病(IgAN)或膜性肾病(MN)患者的的临床病理特征及预后特点。方法:选取2004年1月~2015年12月于南京总医院国家肾脏疾病临床医学研究中心经肾活检确定为抗GBM病合并IgAN患者(合并IgAN组)10例,抗GBM病合并MN患者(合并MN组)5例,与60例单纯抗GBM病患者(单纯抗GBM病组)比较,分析三组患者在临床表现、肾活检病理特征及预后的差异。结果:与单纯抗GBM病组相比,合并IgAN组、合并MN组患者出现无尿者较少,蛋白尿和镜下血尿较多,抗GBM抗体滴度较低,但均无统计学差异。单纯抗GBM病组患者肾损伤程度较重,临床表现为急进性肾炎综合征(RPGN)的比例明显增高(P=0.004),高血压比例高(P=0.002),血肌酐峰值最高(P<0.001),且贫血程度重(P<0.001)。单纯抗GBM病组患者总新月体比例高(P=0.008)且包囊壁断裂比例亦高(P=0.005)。单纯抗GBM组患者需连续肾脏替代治疗比例最高(P=0.008)。单纯抗GBM病组患者1年内进展至终末期肾病比例最高(P<0.001)。结论:合并Ig AN或MN的抗GBM病在临床表现、实验室检查、病理指标及预后均不同于单纯抗GBM病。

Objective： To investigated the differences in clinical characteristics,pathological features and prognosis of anti-glomerular basement membrane（ GBM） disease combined with IgA nephropathy or membranous nephropathy.Methodology： From January 2004 to December 2015,seventy five patients with anti-GBM disease were enrolled into this retrospective study. According to the pathological diagnosis,the patients were divided into three group,including 10 cases of anti-GBM disease with IgA nephropathy（ with Ig AN group）,5 cases of anti-GBM disease with membranous nephropathy（ with MN group） and 60 cases of simple anti-GBM disease（ simple anti-GBM disease group）. The clinical,laboratory and pathological indices were compared among the three groups. Results： In patients of anti-GBM disease with IgAN group and with MN group,they had lower percents of anuria,while more urine protein and microscope hematuria,and a lower level of anti-GBM antibodies,but not statistical significance. Furthermore,the patients in simple anti-GBM disease group were more serious in renal dysfunctions,which represented as highest percents of rapidly progressive glomerulonephritis（ RPGN）（ P= 0. 004）,highest levels of hypertension（ P= 0. 002）,serum creatinine（ SCr,P〈0. 001） and a more serious degree of anemia（ P 〈0. 001）. In pathology,the patients in simple anti-GBM disease group were observed to have the highest percentage of crescents in glomeruli（ P = 0. 008） and Bowman＇s wall fracture（ P = 0. 005）. In the course of treatment,the patients in simple anti-GBM disease group had the highest proportion of continuous renal replacement therapy（ P= 0. 008）. During the follow-up,the patients in simple anti-GBM disease group had the highest percentage of end stage renal disease（ ESRD） in one years（ P 〈0. 001）. Conclusion： Anti-GBM disease with IgA nephropathy or membranous nephropathy is different from simple anti-GBM disease in clinical manifestations, laboratory indices, pathology and prognosis.