抗着丝点抗体阳性的原发干燥综合征临床及实验室特征分析

Clinical characteristics and laboratory features of primary Sj gren's syndrome with positive anticentromere antibody

目的抗着丝点抗体(ACA)阳性的原发干燥综合征(pSS)是干燥综合征(SS)的一个亚型,ACA在pSS中的临床意义尚未明确。文中旨在探讨ACA阳性pSS患者临床表现与实验室检查特征。方法收集大连医科大学附属第一医院风湿免疫科收治的88例pSS患者临床资料,其中ACA阳性患者43例(阳性组)、ACA阴性患者45例(阴性组)。对比各组患者临床表现及实验室指标差异。结果阳性组患者年龄[(63.0±11.1)岁]高于阴性组年龄[(57.7±12.5)岁],差异有统计学意义(P<0.05)。阳性组雷诺现象发生率更高(37.2%vs 15.6%,P<0.05)。阳性组合并原发性胆汁性肝硬化显著高于阴性组(P<0.05)。阳性组的AST、ALP、GGT、IgM水平高于阴性组,IgG和IgA水平低于阴性组,差异有统计学意义(P<0.05)。阳性组患者血清抗SSA、SSB抗体和RF阳性率低(P<0.05),抗线粒体抗体(AMA)阳性率高(P<0.05)。2组间低钾血症、间质性肺炎、肺间质纤维化、肾功能异常、血液系统受累和肿瘤发生率差异均无统计学意义(P>0.05)。结论ACA阳性pSS的临床表现和实验室检查结果均具有特征性,患者年龄大,雷诺现象、肝损害、合并PBC较多见。

Objective Anti-centromere antibody(ACA)-positive primary Sj gren's syndrome(SS)is considered a subtype of SS,and the clinical significance of ACA in SS has not been totally clear.The purpose of this study is to investigate the clinical manifestation and laboratory examination characteristics of primary Sj gren's syndrome(pSS)with anticentromere antibody(ACA).Methods The clinical data of 88 pSS patients admitted to the Department of Rheumatology Immunology of the First Affiliated Hospital of Dalian Medical University were collected,including 43 ACA positive patients(ACA positive group)and 45 ACA negative patients(ACA negative group).The clinical characteristics and laboratory indicators of each group were compared.Results The mean age of pSS patients with ACA positive(63.0±11.1)was higher than that of pSS with ACA negative(57.7±12.5),and in the group of ACA(+)pSS,Renand's phenomenon(RP)was more frequent(P<0.05).The pSS patients with ACA presented a significantly higher prevalence of PBC than those of pSS with ACA negative(P<0.05).Compared with ACA(-)pSS group,the serum levels of AST,ALP,GGT and IgM were higher in ACA(+)pSS group(P<0.05),and the serum levels of IgG and IgA were lower than those of ACA(-)pSS group.In the group of ACA(+)pSS,the positive rates of anti-SSA,anti-SSB antibody and RF were all lower(P<0.05),while the positive rate of anti-mitochondrial antibody(AMA)was higher(P<0.05).While in terms of hypokalemia,interstitial pneumonia,pulmonary fibrosis,abnormal renal function,hematologic involvement and merge tumor,there was no statistical difference(P>0.05).Conclusion The clinical characteristics and laboratory features of pSS patients with positive ACA differ from those with negative antibody.pSS patients with ACA positive were older,they had more Reynolds phenomenon,liver injury and combined PBC.