论文部分内容阅读
目的了解肾功能正常的抗肾小球基底膜(GBM)病的临床和病理特点。方法回顾性分析6例长期保持肾功能正常的抗GBM病患者的临床病理特点,并与同期伴肾功能损害的29例抗GBM病患者进行比较,分析影响预后的因素。结果 35例患者中6例(17.1%)肾功能一直保持正常。5例患者有不同程度肾脏受累,1例仅有肺出血,3例表现为肺肾出血综合征。6例均行肾活检病理检查,免疫荧光:4例表现为IgG 2+～3+沿肾小球毛细血管襻呈线样沉积;光镜差异较大,从肾小球轻微病变到新月体肾炎伴大量毛细血管襻坏死,但大多数患者病变轻微。5例患者给予免疫抑制和(或)血浆置换治疗。6例肾功能正常者与同期肾功能异常的29例抗GBM病相比,贫血轻[血红蛋白(99.67±19.80)比(77.97±20.62)g/L,P=0.024]、抗GBM抗体滴度低[(80.23±85.73)比(224.34±145.79)EU/ml,P=0.027]、肾小球新月体比例低(0.17±0.27比0.58±0.29,P=0.005)。平均随访12～133个月,6例肾功能正常者4例完全治愈,2例遗留少量蛋白尿、镜下血尿。结论肾功能正常的抗GBM病并不少见,多数患者肾脏临床病理轻,预后良好。
Objective To investigate the clinical and pathological features of anti-glomerular basement membrane (GBM) with normal renal function. Methods A retrospective analysis of 6 cases of long-term maintenance of renal function in patients with anti-GBM clinicopathological features, and with the same period with renal dysfunction in 29 cases of anti-GBM patients were compared to analyze the factors that affect the prognosis. Results Of the 35 patients, 6 cases (17.1%) had normal renal function. Five patients had varying degrees of renal involvement, one had only pulmonary hemorrhage, and three showed pulmonary-renal hemorrhage syndrome. 6 cases were performed renal biopsy pathological examination, immunofluorescence: 4 cases showed IgG 2 + ~ 3 along the glomerular capillary loop was deposited; light microscopy vary slightly from mild glomerular lesions to the crescent Nephritis with a large number of capillaries necrosis, but the majority of patients with minor lesions. Five patients were immunosuppressed and / or treated with plasmapheresis. In 6 patients with normal renal function, 29 anemia patients with renal dysfunction had mild anemia [hemoglobin (99.67 ± 19.80) vs (77.97 ± 20.62) g / L, P = 0.024] [(80.23 ± 85.73) vs (224.34 ± 145.79) EU / ml, P = 0.027], and glomerular crescent proportion was lower (0.17 ± 0.27 vs 0.58 ± 0.29, P = 0.005). The patients were followed up for an average of 12 to 133 months. In 6 cases with normal renal function, 4 cases were completely cured, 2 cases left a small amount of proteinuria and microscopic hematuria. Conclusion The anti-GBM disease with normal renal function is not uncommon. Most patients have mild renal pathology and good prognosis.