甲襞微循环检查在抗着丝点抗体阳性原发性干燥综合征的临床价值

Clinical value of nailfold videocapillaroscopy in primary Sjögren's syndrome with anticentromere antibody

目的研究抗着丝点抗体(anticentromere antibody,ACA)阳性的原发性干燥综合征(primary Sjögren's syndrome,pSS)患者甲襞微循环(nailfold videocapillaroscopy,NVC)的特点及意义。方法收集pSS患者53例,其中ACA阳性组22例,ACA阴性组31例。ACA阳性患者除外系统性硬化症(systemic sclerosis,SSc)。两组均行NVC检查并比较各项指标及差异。结果ACA阳性组患者发病年龄较晚[(54.73±6.94)岁vs.(46.94±5.60)岁,P<0.001],易出现雷诺现象(31.8%vs.6.45%,P=0.015),且更易出现肺功能一氧化碳弥散量(diffusing capacity of the lung for carbon monoxide,DLCO)下降(27.3%vs.6.45%,P=0.037)及肺动脉压力(pulmonary artery systolic pressure,PASP)的升高(22.7%vs.3.23%,P=0.027)。ACA阳性组更易出现甲襞毛细血管袢直径增宽[输入袢直径(27.09±14.40)μm vs.(14.45±3.35)μm,P<0.001;输出袢直径(29.32±16.36)μm vs.(15.55±3.61)μm,P<0.001;袢顶直径(35.05±30.23)μm vs.(16.29±3.67)μm,P=0.001]、袢周渗出(轻度渗出22.7%vs.3.23%,中度渗出18.2%vs.3.23%,P=0.047)与出血(22.7%vs.0%,P=0.021)等“非特异性SSc样”NVC异常改变,但两组均未见巨大毛细血管袢。两组患者的NVC参数中,管袢直径、渗出与出血的异常程度与病程、出现雷诺现象、DLCO下降及PASP升高显著相关(P<0.05)。结论ACA阳性pSS是pSS的一种特殊亚型。NVC检查可能有助于ACA阳性pSS患者的预后判断,需密切随访其未来发展为SSc可能。

Objective To observe the significance of nailfold videocapillaroscopy(NVC)in primary Sjogren's syndrome(pSS)with anticentromere antibody(ACA).Methods Fifty-three pSS patients were divided into ACA positive group(n=22)and ACA negative group(n=31).The systemic sclerosis(SSc)patients were excluded from the ACA positive group.NVC was performed and the abnormality of nailfold microcirculation between the two groups were compared.The relations between NVC findings and clinical laboratory parameters were compared.Results The average age of onset was elder in patients with ACA than in patients without ACA[(54.73±6.94)yr vs.(46.94±5.60)yr,P<0.001],also the prevalence of Raynaud's phenomenon was higher among ACA positive patients(31.8%vs.6.45%,P=0.015).Furthermore,ACA positive patients had a higher prevalence of diffusing capacity of the lung for carbon monoxide(DLCO)decreasing(27.3%vs.6.45%,P=0.037)and pulmonary artery systolic pressure(PASP)elevating(22.7%vs.3.23%,P=0.027)compared to ACA negative patients.Patients with ACA have more enlarged of the capillary diameter[the input capillary diameter(27.09±14.40)μm vs.(14.45±3.35)μm,P<0.001;the output capillary diameter(29.32±16.36)μm vs.(15.55±3.61)μm,P<0.001;the apical diameter(35.05±30.23)μm vs.(16.29±3.67)μm,P=0.001].Compared with those without ACA,patients with ACA presented a higher prevalence of“non-specific SSc-like”changes,such as exudation(the mild 22.7%vs.3.23%,the moderate 18.2%vs.3.23%,P=0.047)and bleeding(22.7%vs.0%,P=0.021).No giant capillary loops were seen in both groups.Among the NVC parameters of the two groups,the degree of abnormality of the capillary diameter,the exudation and bleeding were significantly related to the course of the disease,Raynaud's phenomenon,decrease of DLCO and elevated of PASP(P<0.05).Conclusion ACA positive pSS is an unique subtype of pSS.NVC examination can be helpful for predicting prognosis of pSS patients with ACA positive and it's necessary to follow up closely for their future development of SSc.