76例淀粉样变性肾损害的临床病理特点
| 作者: |
1向流霞,
1袁曙光,
1张新民,
1朱雪婧,
1张三勇,
1杨淡昳,
1许向青,
1刘虹,
1孙林
1 中南大学湘雅二医院肾内科，中南大学肾脏病研究所，长沙 410011 |
| 通讯: |
袁曙光
Email: shuguangy@csu.edu.cn 张新民 Email: liuxiainthesky@126.com |
| DOI: | 10.3978/j.issn.2095-6959.2018.08.013 |
| 基金: | 国家自然科学基金(81300600)；湖南省自然科学基金(2018JJ3728，2018JJ2596)；中南大学自由探索计划(2012QNZT146)。 |
摘要
目的：分析淀粉样变性肾损害的临床表现及肾脏病理特点，为临床诊治提供依据，减少误诊和漏诊。方法：回顾分析2011年8月至2017年8月湘雅二医院肾内科经肾活检诊断为淀粉样变性肾损害76例患者的临床和病理资料。结果：76例淀粉样变性肾损害患者年龄(55.3±10.4)岁，93.4%的患者年龄在40岁以上，男女比为1.5:1。61%的患者有2个以上系统损害，77.6%的患者表现为典型的肾病综合征。免疫球蛋白轻链型(amyloid immunoglobulin light chain，AL)淀粉样变性肾损害75例，其中7例伴发多发性骨髓瘤(multiple myeloma，MM)，血清淀粉样蛋白A型淀粉样变性肾损害(amyloid A，AA)1例。66.2%的患者经血/尿免疫固定电泳检出单克隆免疫球蛋白轻链，76例行免疫球蛋白轻链λ，κ免疫荧光检查，λ链阳性为主73.7%，κ链阳性为主13.2%，两者比值约为6:1。透射电镜检查可见淀粉样蛋白主要沉积于肾小球系膜区、基底膜及肾小动脉壁。结论：肾淀粉样变在蛋白尿患者中并非少见，患者年龄并不局限于40岁以上，常累及多系统，临床表现多样，易误诊。肾活检光镜下的典型病理学改变结合刚果红染色可确诊，透射电镜检查可帮助早期诊断。
关键词：
淀粉样变性肾损害；超微病理诊断；病理分型
Clinicopathological features of renal impairment in 76 cases with amyloidosis
CorrespondingAuthor: YUAN Shuguang Email: shuguangy@csu.edu.cn
DOI: 10.3978/j.issn.2095-6959.2018.08.013
Foundation: This work was supported by the National Natural Science Foundation (81300600), the Natural Science Foundation of Hunan Province (2018JJ3728
Abstract
Objective: To investigate the clinical and pathological features of renal amyloidosis in order to provide assistance for clinical diagnosis and treatment. Methods: A retrospective analysis was performed on 76 patients with renal amyloidosis in our hospital from August 2011 to August 2017. Results: The average age of these patients was (55.3±10.4) years old; 93.4% of patients were over 40 years old. The ratio of male to female was 1.5:1; 61% of patients had two or more systems involvement; 77.6% of patients typically present with nephrotic syndrome (NS). Seventy-five cases show amyloid immunoglobulin light chain (AL) positive, including 7 cases with multiple myeloma (MM), and one case show serum amyloid A (AA) positive; 66.2% of patients were found to have monoclonal light chain in serum or urine by immunofixation electrophoresis (IFE); 73.7% of 76 patients was λ-chains positive, 13.2% was κ-chains positive, the ratio was 6:1. Under electron microscopy (EM), the amyloid fibrils were randomly distributed in the mesangium, along glomerular basement membrane (GBM) and at the arteriolar wall. Conclusion: Amyloidosis nephropathy mainly occurs in middle-aged patients with heavy proteinuria, and often involves multiple systems, with diverse clinical presentation, leading to misdiagnose. Renal biopsy is the important approach to confirm the diagnosis by Congo red staining, and EM observation play a crucial role in the diagnosis of early renal amyloidosis.
Keywords：
renal amyloidosis; ultrapathological diagnosis; pathological classification