20例肾淀粉样变的临床与病理特点分析

Analysis of clinical and pathological characteristics of 20 patients with renal amyloidosis

目的:分析肾淀粉样变的临床与病理特点,以减少误诊和漏诊,提高临床诊治水平。方法:回顾性分析2005年1月至2009年8月在中南大学湘雅二医院肾内科经活检诊断为肾淀粉样变的20例患者的临床和病理资料。结果:20例中95%的患者年龄在40岁以上,55%的患者有2个以上系统损害,80%表现为典型的肾病综合征。肾活检HE染色下肾小球系膜及毛细血管袢周边可见均一的淀粉样物质呈块状沉积,亦可见于间质、血管及肾小管壁。高锰酸钾氧化刚果红染色示AL型18例,AA型2例。AL型与AA型病理改变类似,但前者可见免疫球蛋白轻链κ和λ沉积。结论:肾淀粉样变在中老年蛋白尿患者中并非少见,临床表现有时缺乏特异性,肾活检光镜下的典型病理学改变结合刚果红染色可确诊。对于疑似患者应尽快行肾活检及刚果红染色,以免误诊与漏诊。

Objective To analyze the clinical and pathological characteristics of renal amy-loidosis,to decrease the chance of misdiagnosis and missed diagnosis,and to improve the diagnosis accuracy and the therapeutical effect.Methods We retrospectively analyzed the clinical and pathological data of 20 patients with renal amyloidosis,who were hospitalized in the Second Xiangya Hospital from Jan.2005 to Aug.2009 and were diagnosed as renal amyloidosis by renal biopsy.Results Of the 20 patients,19（95%） were over 40 years old,55% patients suffered more than 2 systems injuries,and 80% patients displayed the typical nephrotic syndrome.Brick-red homogeneous amyloid deposition could be seen in glomerular mesangial cell and capillary membrane loop after HE staining as well as in the interstitials,the blood vessels,and the tubular wall.Eighteen patients were AL type and potassium permanganate test was positive.Another 2 were AA type and potassium permanganate test was negative.The pathological characteristics of AL type was similar to that of AA type.Immunoglobulin light chain κ and λ could be detected in AL type but not in AA type.Conclusion Renal amyloidosis is common in elderly patients.The clinical manifestations of renal amyloidosis are not always specific.It could be made the final diagnosis through examining the typical pathophysiolgical changes by renal biopsy combined with Congo red staining.Suspected patients should be performed with renal biopsy and Congo red staining as soon as possible to avoid misdiagnosis and missed diagnosis.