抗着丝点抗体阳性原发性干燥综合征的临床特点

目的 通过分析抗着丝点抗体(ACA)阳性原发性干燥综合征(pSS)患者的临床特点,提高对本病的诊治水平.方法 回顾性分析北京协和医院风湿免疫科收治的60例ACA阳性pSS患者(ACA阳性组)的临床资料,并与同期收治的139例ACA阴性pSS者(ACA阴性组)相比较.结果 (1)ACA阳性组发病年龄晚于ACA阴性组(48±11)岁比(41±12)岁,P=0.000],而2组在性别构成、口干、眼干、唇腺活检阳性率差异元统计学意义.(2)肝脏受累是ACA阳性组患者最常见的腺外脏器损害,其发生率远高于ACA阴性组(68.3%比37.0%,P=0.000),而肾脏受累(13.3%比30.9%,P=0.009)、神经系统受累(1.7%比11.5%,P=0.025)、高γ球蛋白血症的发生率(20.8%比45.7%,P=0.002)均低于ACA阴性组.(3)2组抗核抗体阳性率无差异,但ACA阳性组以散点型免疫荧光表型最为常见(占61.7%).与ACA阴性组相比,ACA阳性组中抗SSA抗体、抗SSB抗体、抗U1 RNP抗体的阳性率减低(P＜0.05),而抗线粒体抗体及其M2亚型的阳性率增高(P＜0.05).(4)ACA阳性组死亡5例,其中3例死于胃食管静脉曲张破裂出血.结论 对ACA阳性pSS者应警惕肝脏受累.由于ACA阳性pSS在发病年龄、腺外器官受累、免疫指标、自身抗体谱及预后方面均不同于经典pSS,故可能是pSS的一种独特亚型.

A clinical analysis of primary Sj(o)gren's syndrome with anticentromere antibodies

Objective To investigate the clinical manifestations, immunological features and prognosis of primary Sj(o)gren's syndrome(pSS) with anticentromere antibodies(ACA). Methods Sixty pSS patients with ACA in our hospital between 1985 and 2006 were screened retrospectively and compared with those without ACA. Results The mean age at the onset of pSS with ACA was higher than that of those without ACA(48±11)yr vs(41±12)yr,P=0.000].There was no difference in sex ratio,dry mouth.dry eyes and positive salivary gland biopsy between the two groups(P＞0.05).Compared with those without ACA,patients with ACA presented a higher prevalence of liver involvement(68.3%vs 37.0%,P=0.000),while a lower prevalence of renal involvement(13.3%vs 30.9%,P=0.009), neuropathy(1.7% vs 11.5%,P=0.025)and hypergammaglobulinemia(20.8%vs 45.7%,P=0.002). The difference was not significant between the two groups in Raynaud's phenomenon,articular involvement,myositis,hematologic involvement,lung involvement,and thyroiditis. While both groups showed the same prevalence of antinuclear antibody(ANA),the patterns of ANA-IF were different and the discrete speckled pattern was the most freqent in patients with ACA and occurred in 61.7%. Different from those without ACA,patients with ACA presented a lower prevalence of anti-SSA,anti-SSB, rheumatoid factor, and antiU1 RNP,while showed a higher prevalence of antimitochondrial antibodies(AMA) and AMA-M2. The most frequent cause of death was the complications associated with cirrhosis,notably bleeding varices(3/5 cases). Conclusion Patients with ACA present a high risk of liver involvement. Because of the remarkable difference in the mean age of disease onset and also differences in systemic damage, immunological and antibody features, pSS with ACA may be a special subtype of pSS.