国人190例免疫球蛋白轻链型淀粉样变性病临床与病理的相关性分析

目的：探讨免疫球蛋白轻链（AL）型淀粉样变性病肾组织病理改变与肾活检时临床表现的相关性。方法研究对象为1990年1月至2011年12月在北京大学第一医院经肾活检诊断的AL型肾淀粉样变性病患者共190例。对淀粉样物质在肾小球、肾血管和肾间质沉积的程度进行半定量评分,并进行临床病理特点的相关性分析。结果（1）肾小球淀粉样变程度与尿蛋白量呈正相关（rs＝0.298,P＜0.001）;（2）肾小球淀粉样变程度重的患者肾功能不全的发生率高（χ2＝34.027,P＜0.001）;（3）肾血管淀粉样变程度越重,肝脏受累、心脏受累的发生率越高（χ2＝15.916,P＜0.001;χ2＝6.189,P＝0.014）;（4）AL-κ患者肝脏受累的发生率和心脏受累的发生率都显著高于AL-λ患者（75%vs.24.4%,χ2＝16.057,P＜0.001;72.7%vs.34.2%,χ2＝5.757,P＝0.020）;前者的肾血管淀粉样变程度评分亦显著高于后者[3.5（3～4）vs.2（1～3）, Z＝-3.118,P＝0.002]。结论 AL型肾淀粉样变性病患者肾组织中肾小球淀粉样变的程度与确诊时的蛋白尿水平和肾功能相关,肾血管淀粉样变的程度与心脏和肝脏受累相关。AL-κ患者肾血管淀粉样变程度重,容易发生肝脏和心脏受累。

Associations between pathological features and clinical manifestations of immunoglobulin fight-chain renal amyloidosis

Objective To investigate the relationship between clinical manifestations and pathological features in patients with renal immunoglobulin light-chain amyloidosis（AL）. Methods A series of 190 patients with biopsy-proven AL renal amyloidosis were collected from our institute between January 1990 and December 2011. The extent of amyloid deposition in glomeruli（GA）, blood vessels（VA） and tubulointerstitium（IA） were evaluated semiquantitatively. The correlations between the clinical manifestations and pathological features were analyzed statistically. Results The extent of glomerular amyloid deposition was positively correlated with proteinuria level（rs=0.298, P〈0.001）. Patients with advanced glomerular amyloid deposition（GA=5） had a much higher incidence of renal insufficience than others. Cardiac involvement and hepatic involvement were all positively correlated with the degree of vascular amyloid deposition in renal biopsy（χ2=15.916, P〈0.001; χ2=6.189, P=0.014）. The incidences of hepatic involvement and cardiac involvemen were all much higher in patients with AL-κthan in patients with AL-λ（75%vs. 24.4%,χ2=16.057, P〈0. 001;72.7%vs. 34.2%,χ2=5.757, P=0.020）. Patients with AL-κ had higher VA than patients with AL-λ [3.5（3-4） vs. 2（1-3）, Z=-3.118, P=0.002]. Conclusions The degree and localization of amyloid deposits in the kidney of AL patients were associated with the degree of proteinuria and renal insufficiency, as well as hepatic and cardiac involvement. Patients with AL-κwere more likely to have hepatic and cardiac involvement and severe vascular amyloid deposition than AL-λ.