中文摘要：

目的研究原发性干燥综合征（pSS）并淀粉样变患者外周血血清游离轻链（sFLC）和细胞因子水平的特点及环磷酰胺治疗对其的影响。方法检测9例初诊的pSS并淀粉样变患者治疗前和环磷酰胺治疗3、6、12个月时外周血sFLC、肿瘤坏死因子（TNF）-α、转化生长因子（TGF）-β、白细胞介素（IL）．4水平，治疗前后指标变化采用重复测量设计的方差分析。结果pSS并淀粉样变患者sFLC中均为入型，未发现K型。治疗后12个月sFLC水平（22±42）mg／L低于治疗前（180±15）mg，L（P〈O．05）。pSS并淀粉样变患者环磷酰胺治疗后3个月细胞因子水平出现下降，治疗后12个月TNF．仪、TGF．B。、IL．4水平分别为（24．0±1．4）、（58±5）、（38．8±3．2）mg／L，均较治疗前[（31．1±2．0）、（65±5）、（61．2±3．8）ng／L]下降，差异有统计学意义（P〈0．05）。结论pSS并淀粉样变患者外周血sFLC水平和细胞因子水平明显异常，治疗后下降，提示细胞因子可能在其发病机制中起重要作用；环磷酰胺可能有较好的治疗效果。

英文摘要：

Objective To study the characteristics of the serum free light chain （sFLC） and cytokines levels and the effects of cyclophosphamide （CTX） on patients with primary Sjogren＇s syndrome （pSS） complicated with amyloidosis. Methods Nine patients of pSS with amyloidosis who were admitted to Department of Rheumatology of the hospital from Jan 2005 to Mar 2011 were selected. The serum levels of sFLC and cytokines TNF-α, TGF-β, IL-4 were measured before and 3, 6, 12 months after the treatment respectively. Meanwhile, Twelve patients with pSS without complications were selected as controls. Repeated measures analysis of variance was used for data analysis. Results The sFLC in serum of pSS patients with amyloidosis was λtype, the κtype was not found. The level of serum sFLC after 12 months treatment [（22±42） mg/L] was significantly lower than before treatment [（180±15） mg/L]. The levels of TNFα, TGF-β, IL-4 after 12 months treatment [（24.0±1.4）, （58±5）, （38.8±3.2） ng/L, respectively] were significantly lower than before treatment [（31.1±2.0）, （65±5）, （61.2±3.8） ng/L, respectively]. Conclusion The levels of serum sFLC and cytokines are abnormally increased in patients of pSS with amyloidosis. After treatment, the levels of cytokines are decreased initially which indicates that these eytokines may play a key role in the pathogenesis of pSS. CTX therapy may lead to better efficacy and more favorable prognosis.