Abstract：
We evaluated the prevalence of systemic sclerosis (SSc)-related autoantibodies and their clinical significance and compared the sensitivity of two line immunoblot assays on a prospective study group of 96 Polish SSc patients (ACR-EULAR 2013 criteria) whose sera were assessed by indirect immunofluorescence (HEp-2 and monkey liver) and line immunoblot assays: ANA Profile 3 and Systemic Sclerosis Profile by EUROIMMUN (Lübeck, Germany). Organ involvement was evaluated according to the EUSTAR Minimal Essential Data Set. The following autoantibodies’ prevalence was found: Scl-70 (36%), Ro-52 (28%), CENP-B (22%), CENP-A (20%), PM-Scl-75 (20%), PM-Scl-100 (14%), fibrillarin (7%), Th/To (7%), RNA polymerase III 11 kDa (5%), RNA polymerase III 155 kDa (3%), PDGFR (3%), NOR-90 (2%), and Ku (1%). Significant associations between the autoantibodies’ presence and organ involvement were found: ATA (dcSSc > lcSSc, less prevalent muscle weakness), Ro-52 (gangrene, DLCO < 60), CENP-B and A (lcSSc > dcSSc, normal CK), CENP-B (rarer digital ulcers and joint contractures), PM-Scl-100 and 75 (PM/SSc overlap, CK increase, muscle weakness, muscle atrophy), PM-Scl-100 (dcSSc unlikely), PM-Scl-75 (lung fibrosis), fibrillarin (muscle atrophy, proteinuria, conduction blocks, palpitations), Th/To (proteinuria, arthritis, muscle weakness, and rarer esophageal symptoms), RNA Polymerase III 11 kDa (arterial hypertension, renal crisis), RNA polymerase III 155 kDa (renal crisis), and PDGFR (dcSSc, tendon friction rubs). Additionally, the Systemic Sclerosis Profile was significantly more sensitive in detecting SSc-related autoantibodies than ANA Profile 3 (p = 0.002). In conclusion, individual autoantibodies associated with specific characteristics of SSc.
摘要：
我们评估了系统性硬化症（SSc）相关自身抗体的患病率及其临床意义，并比较了96名波兰SSc患者（ACR-EULAR2013标准）的前瞻性研究组的两线免疫印迹测定法的敏感性，这些患者的血清通过间接免疫荧光（HEp-2和猴肝）和线免疫印迹测定法：ANA谱3和EUROIMMUN的系统性硬化症谱（吕贝克，德国)。根据EUSTAR最小必要数据集评估器官受累情况。发现以下自身抗体患病率：Scl-70（36%），Ro-52（28%），CENP-B（22%），CENP-A（20%），PM-Scl-75(20%),PM-Scl-100（14%），纤维素（7%），Th/至（7%），RNA聚合酶III11kDa（5%），RNA聚合酶III155kDa（3%），PDGFR（3%），NOR-90（2%），和Ku（1%）。发现自身抗体的存在和器官受累之间存在显著关联：ATA(dcSSc>lcSSc，不太普遍的肌肉无力)，Ro-52(坏疽,DLCO<60)，CENP-B和A(lcSSc>dcSSc,正常CK)，CENP-B（罕见的手指溃疡和关节挛缩），PM-Scl-100和75(PM/SSc重叠，CK增加,肌肉无力,肌肉萎缩)，PM-Scl-100（dcSSc不太可能），PM-Scl-75（肺纤维化），原纤（肌肉萎缩，蛋白尿,传导阻滞,心悸)，Th/To(蛋白尿，关节炎,肌肉无力,和罕见的食道症状)，RNA聚合酶III11kDa（动脉高血压，肾危象)，RNA聚合酶III155kDa（肾危象），和PDGFR(DCSSc，肌腱摩擦摩擦)。此外,在检测SSc相关自身抗体方面,系统性硬化症谱比ANA谱3更敏感(p=0.002).总之,与SSc的特定特征相关的个体自身抗体。
