CAJ | 학술논문

目的研究肾组织中缺乏IgG沿肾小球毛细血管袢线样沉积的抗肾小球基底膜(GBM)病患者的临床、病理及预后特点.方法选取北京大学第一医院肾内科1991年至2008年确诊的抗GBM病患者93例,其中40例肾脏病理中冰冻切片直接免疫荧光检查缺乏IgG沿肾小球毛细血管袢沉积的患者为研究组(A组),53例肾组织中有IgG沿肾小球毛细血管袢线样沉积的经典抗GBM病患者为对照组(B组),比较两组患者的临床、病理和预后的差异.结果两组患者在性别、年龄、咯血、少尿或无尿和肉眼血尿的发生率、蛋白尿水平、贫血程度、循环中抗GBM抗体百分结合率、ANCA阳性率、肾脏病理肾小球中新月体的比例及组成成分、患者的生存率以及肾脏预后等方面,差异均无统计学意义(均P＞ 0.05).肾组织中缺乏IgG沉积的患者,从起病到确诊所需的时间较长(68 d比36 d,P=0.013)；确诊时的血肌酐水平较低(716.0μmol/L比896.8 μmol/L,P=0.027).其中4例患者肾组织石蜡切片行直接免疫荧光检查可以见到IgG沿GBM呈线样沉积.结论肾脏病理冰冻切片直接免疫荧光缺乏IgG线样沉积的抗GBM病患者,与经典抗GBM病患者相比,肾脏病变进展较慢,但其他临床及病理表现并无显著差异,肾脏预后及患者存活率亦无显著差异.因此,临床上应尽早检测血清抗GBM抗体,早期给予血浆置换治疗,以改善预后.
목적 연구신조직중결핍IgG연신소구모세혈관번선양침적적항신소구기저막(GBM)병환자적림상、병리급예후특점.방법 선취북경대학제일의원신내과1991년지2008년학진적항GBM병환자93례,기중40례신장병리중빙동절편직접면역형광검사결핍IgG연신소구모세혈관번침적적환자위연구조(A조),53례신조직중유IgG연신소구모세혈관번선양침적적경전항GBM병환자위대조조(B조),비교량조환자적림상、병리화예후적차이.결과 량조환자재성별、년령、각혈、소뇨혹무뇨화육안혈뇨적발생솔、단백뇨수평、빈혈정도、순배중항GBM항체백분결합솔、ANCA양성솔、신장병리신소구중신월체적비례급조성성분、환자적생존솔이급신장예후등방면,차이균무통계학의의(균P＞ 0.05).신조직중결핍IgG침적적환자,종기병도학진소수적시간교장(68 d비36 d,P=0.013)；학진시적혈기항수평교저(716.0μmol/L비896.8 μmol/L,P=0.027).기중4례환자신조직석사절편행직접면역형광검사가이견도IgG연GBM정선양침적.결론 신장병리빙동절편직접면역형광결핍IgG선양침적적항GBM병환자,여경전항GBM병환자상비,신장병변진전교만,단기타림상급병리표현병무현저차이,신장예후급환자존활솔역무현저차이.인차,림상상응진조검측혈청항GBM항체,조기급여혈장치환치료,이개선예후.
Objective To investigate the clinical and pathological features of patients with anti-glomerular basement membrane (GBM) disease lacking linear IgG deposition along GBM on renal biopsy. Method Ninety-three patients with anti-GBM disease were collected in our hospital from 1991 to 2008,with 40 patients presenting negative linear IgG deposition along GBM on renal biopsy by direct immunofluorescence (group A) and 53 patients presenting classical linear IgG deposition along GBM (group B).The clinical manifestation,pathological presentation and prognosis were compared between two groups. Results Between two groups,there were no significant differences in gender,age,hemoptysis,oliguria or anuria,gross hematuria,proteinuria,anemia,ANCA positivity,level of circulating anti-GBM antibodies,the percentage of crescent formation in glomeruli and patient outcomes (P＞0.05).Patients in group A were diagnosed significantly later than patients in group B (68 d vs 36 d,P=0.013) and serum creatinine was significantly lower at diagnosis (716.0 μmol/L vs 896.8 μmol/L,P=0.027).Direct immunofluorescence was performed on the paraffin-embedded renal sections from four patients in group A,and all of them revealed positive linear IgG deposition along GBM. Conclusions Patients with circulating anti-GBM antibodies but withont IgG deposition along GBM present slower progress of renal injury,but same clinical,pathological and prognostic features as those with classical anti-GBM disease.Serum anti-GBM antibodies should be prescribed earlier to the suspected patients,and the diagnosed patients should be treated with plasmapheresis and extensive immunosuppression to improve prognosis.