目标:抗CENP-B(ACA),抗拓扑异构酶I(ATA)和抗RNA聚合酶III(RP3)自身抗体被纳入2013年SSc-ACR/EULAR分类标准.当其它自身抗体为阴性时,检测其它自身抗体是令人感兴趣的。此外,我们想知道IgA同种型是否可能在SSc中起作用。该研究的目的是评估ACA的患病率,ATA,IgG和IgA同种型的RP3和Ro52自身抗体,并描述它们与SSc患者队列中临床表现的关联。
方法:来自符合2013ACR/EULAR分类标准的97例SSc患者的样本,包括50名献血者,并测试了ACA的IgA和IgG同种型,ATA,FEIA的RP3和Ro52。
结果:相同特异性的IgG+IgA同种型的患病率为62.5%,82.6%,80.0%,36.8%,对于ACA,ATA,分别为RP3和Ro52。分离的IgG以35.4%存在,13.0%,20.0%和42.1%的患者为ACA,ATA,分别为RP3和Ro52。只有6名患者因独特的特异性而被分离为IgA。临床上,ILD倾向于与ATA-IgG和ATA-IgG+IgA相关,ACA-IgG+IgA的毛细血管扩张和ACA-IgA的关节炎。的确,ATA-IgG患者中数字溃疡更为常见.
结论:大多数患者出现ACA,ATA,或除IgG外的IgA同种型的RP3自身抗体。关于临床相关性,Ro52-IgG+IgA和ACA-IgG有向sineSSc表型的趋势,而ACA-IgG+IgA为lcSSc表型。因此,如果确认,ACA-IgA的测定可以为根据皮肤表型对患者进行分层提供工具.
OBJECTIVE: Anti-CENP-B (ACA), anti-topoisomerase I (ATA) and anti-RNA polymerase III (RP3) autoantibodies are included in the 2013 SSc-ACR/EULAR classification criteria. The detection of additional autoantibodies is of interest when those are negative. Additionally, we wonder if the IgA isotype might play a role in SSc. The aims of the
study were to assess the prevalence of ACA, ATA, RP3, and Ro52 autoantibodies of IgG and IgA isotype and to describe their association with clinical manifestations in a cohort of patients with SSc.
METHODS: Samples from 97 patients with SSc fulfilling the 2013 ACR/EULAR classification criteria, and 50 blood donors were included and tested for IgA and IgG isotypes of ACA, ATA, RP3, and Ro52 by FEIA.
RESULTS: The prevalence of IgG+IgA isotypes for the same specificity was 62.5%, 82.6%, 80.0%, 36.8%, for ACA, ATA, RP3 and Ro52, respectively. Isolated IgG was present in 35.4%, 13.0%, 20.0% and 42.1% of patients for ACA, ATA, RP3 and Ro52, respectively. Only six patients were isolated IgA for a unique specificity. Clinically, ILD tended to be associated with ATA-IgG and ATA-IgG+IgA, telangiectasias with ACA-IgG+IgA and arthritis with ACA-IgA. Indeed, digital ulcers were more frequent in ATA-IgG patients.
CONCLUSIONS: Most of the patients presented ACA, ATA, or RP3 autoantibodies of IgA isotype in addition to IgG. Regarding clinical relevance, Ro52-IgG+IgA and ACA-IgG had a tendency towards sineSSc phenotype, while ACA-IgG+IgA to lcSSc phenotype. Thus, if confirmed, the determination of ACA-IgA could provide a tool to stratify patients according to the cutaneous phenotype.