肾脏原发系统性轻链型淀粉样变患者的临床特征和生存分析

Clinical features and survival analysis of light-chain amyloidosis associated renal disease

目的 总结肾脏原发系统性轻链型淀粉样变患者的临床病理特征,探讨影响预后的因素.方法 回顾性分析25例肾脏原发系统性轻链型淀粉样变患者的临床资料,随访其生存情况.结果 25例肾脏原发系统性轻链型淀粉样变患者,中位年龄57（37～69）岁,游离λ轻链型占绝大多数（88%,22/25）.突出症状为大量蛋白尿、肾病综合征伴顽固性水肿,尿蛋白排泄以白蛋白为主.肾脏病理病变主要累及肾小球系膜区局灶性或弥漫性无细胞性明显增宽.25例患者诊断后中位生存时间24.4个月,1、2和3年总生存率分别为（65±10）%、（46±12）%和（15±12）%.明确诊断时单独肾脏淀粉样变14例,淀粉样变同时累及其他脏器组织11例,两者诊断后中位生存时间分别为24.7个月和16.4个月,差异有统计学意义（P=0.03）.Cox回归单因素分析表明：年龄、性别、骨髑浆细胞比例、白蛋白和血红蛋白水平与预后无关（P＞0.05）,肾功能损害和淀粉样变同时累及其他脏器与预后相关（P＜0.05）,其中同时有心脏病变很可能与预后呈负相关（P=0.06）,进一步Cox逐步回归多因素分析表明肾功能损害是影响肾脏原发系统性轻链型淀粉样变患者预后的一个独立危险因素（P＜0.05）.结论 肾功能损害是影响预后的独立高危因素,同时联合有其他脏器尤其是心脏淀粉样变患者预后差.

Objective To analyze the clinical pathology features of light-chain amyloidosis associated renal disease,and investigate the survival influential factors. Method From January 1998 to March 2009,25 patients with light-chain amyloidosis associated renal disease were reviewed and followed up.Results Of the 25 patients with light-chain amyloidosis associated renal disease,median age was 57（37-69） years old and lamda light-chain predominated （88% ,22/25）. Heavy proteinuria and nephrotic syndrome with peripheral edema were typical clinical presentations. Renal biopsy showed that amyloid deposition of light-chain amyloidosis associated renal disease involved the glomeruh mostly, with mesangial area widening. Median survival of all patients was 24.4 months after diagnosis. The estimated 1,2,3 year survival rate was （65 ± 10 ）%, （46 ± 12 ）% and （15 ± 12 ）% respectively. There was significant difference in median survival between the two groups （24.7 months in the group of 14 patients with isolated kidney affected,16.4 months in the group of 11 patients with kidney and other organs involved,P = 0.03）. By univariate analysis, kidney associated with other organs amyloidosis and renal dysfunction were relevant to prognosis （P 〈 0.05） and heart involvement was probably relevant （P = 0.06）,whereas sex,age,plasma cell ratio,serum albumin level and hemoglobin level had no relation（P〉 0.05 ）. Multivariate analysis revealed that renal dysfunction at the time of diagnosis was a significant and independent prognostic factor for survival （P 〈0.05）. Conclusions Renal dysfunction at the time of diagnosis is the best predictor of survival. The presence of amyloidosis in organs other than the kidney, such as advanced cardiac amyloidosis, predicts a poor survival.