新疆地区抗着丝点抗体阳性原发性干燥综合征的临床特点

Clinical analysis of primary Sjgren's syndrome with anticentromere antibodies in Sinkiang

目的探讨新疆地区抗着丝点抗体(ACA)阳性的原发性干燥综合征(pSS)患者的血清学和临床特征。方法对我院20例抗ACA抗体阳性的原发性干燥综合征和66例抗ACA抗体阴性的原发性干燥综合征,进行回顾性临床病例资料分析比较。结果 2组在一般资料上,病程、年龄、族别、眼干、口干、龋齿、唇腺活检(阳性率)上,差异无统计学意义(P>0.05)。与抗ACA抗体阴性的干燥综合征患者相比,抗ACA抗体阳性的干燥综合征患者血清中抗SSA/SSB抗体阳性率低、γ-球蛋白和IgG水平不高(P<0.05),抗ACA抗体阳性的干燥综合征患者,临床表现中更容易出现乏力、气短、雷诺现象和指/趾端溃疡(P<0.05),在本研究中还发现更易出现肺间质纤维化和肺动脉高压(P<0.05)。结论抗ACA抗体阳性的干燥综合征是原发性干燥综合症的特殊临床亚型。

In order to describe the clinical manifestations and serological features of primary Sjgren＇s syndrome（pSS） with anticentromere antibodies（ACA） in Xin Jiang,Twenty pSS patients with ACA in our hospital from 2005 to 2010 were screened retrospectively and compared with sixty-six pSS patients without ACA.Cases and controls did not differ in mean age,duration or in prevalence of ethnical population,decayed tooth,dry mouth,dry eyes and positive salivary biopsy（P 0.05）.Cases showed lower prevalence of gamma globulinemia,anti-SSA/SSB antibodies,immunoglobulin G（IgG） levels compared to ACA negative patients（P 0.05）,but there was no difference between cases and controls in positive rheumatoid factor（RF）,antinuclear antibodies（ANA）,antimitochondrial antibody（AMA）,antimitochondrial-M2 antibody（AMA-M2）,antiU1RNPantibody,C3 complement levels and C4 complement levels（P 0.05）.The predominant clinical features among ACA positive patients were higher prevalence of Raynaud＇s phenomenon,fatigue,dyspnoea and digital ulcers（P 0.05）.Furthermore,ACA positive patients had a higher prevalence of lung fibrosis,pulmonary arterial hypertension（PAH） and diffusing capacity for carbon monoxide（DLCO） decreasing compared to ACA negative patients（P 0.05）.Our analysis indicated that ACA positive pSS is a unique subtype of pSS and demonstrates the clinical difference between ACA positive pSS and ACA negative pSS.