中文摘要：

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（68d比36d，P=0．013）；确诊时的血肌酐水平较低（716．0μmol／L比896．8μmol／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。

英文摘要：

Objective To investigate the clinical and pathological features of patients with antiglomerular 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 wassignificantly 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.