Rheumatology Research Center, Firoozgar Hospital Tehran, Tehran University of Medical Sciences, Tehran, Iran.
Rheumatol Int. 2013 Aug;33(8):1943-50. doi: 10.1007/s00296-013-2668-5. Epub 2013 Jan 24.
To evaluate demographic, clinical and laboratory features associated with scleroderma-specific auto-antibodies. Sera of 100 patients with systemic sclerosis (SSc) were analyzed by an indirect immunofluorescence technique with HEp-2 cells as a substrate. Specific ANA such as anti-centromere antibodies (ACA), anti-topoisomerase (TOPO), anti-RNA polymerase III (Pol 3), anti-U3-RNP (U3-RNP), anti-Th/To (Th/To) and anti-PM/Scl (PM/Scl) were detected by line immunoassay and anti-U1-RNP (U1-RNP) by ELISA. Frequency of clinical features associated with a specific antibody group was reported cumulatively over the follow-up period. Frequency of specific clinical features was compared across the two disease subtype including limited cutaneous (lcSSc) or diffuse cutaneous (dcSSc) as well as the auto-antibody groups. Ninety-four percent of patients were ANA positive with significant higher skin score, Raynauds and digital ulcer/gangrene. Anti-TOPO was detected in 71% of all patients, in 90.5% of dcSSC and in 65.8% of lcSSc. Anti-TOPO was significantly associated with dcSSc, higher skin score, digital ulcer/gangrene, pulmonary fibrosis, DLCO <70%. U1-RNP antibody was associated with lower fibrosis in lung. ACA was positive in 7% of patients and exclusively in those with lcSSc. We did not find association between gender and presence of auto-antibodies. Anti-TOPO antibody had a high prevalence in contrast to low prevalence of ACA antibody. There were no differences in clinical subtypes of the disease in patients with positive anti-TOPO and positive ACA. Differences in prevalence of auto-antibodies are suggestive of further genetic study.
评估与硬皮病特异性自身抗体相关的人口统计学、临床和实验室特征。采用间接免疫荧光技术（以 HEp-2 细胞为底物）分析 100 例系统性硬皮病（SSc）患者的血清。通过线免疫分析检测特定的 ANA，如抗着丝点抗体（ACA）、抗拓扑异构酶（TOPO）、抗 RNA 聚合酶 III（Pol 3）、抗 U3-RNP（U3-RNP）、抗 Th/To（Th/To）和抗 PM/Scl（PM/Scl），抗 U1-RNP（U1-RNP）则通过 ELISA 检测。在随访期间，报告与特定抗体组相关的临床特征的累积频率。比较两种疾病亚型（局限性硬皮病[lcSSc]或弥漫性硬皮病[dcSSc]）以及自身抗体组之间特定临床特征的频率。94%的患者 ANA 阳性，皮肤评分、雷诺现象和手指溃疡/坏疽显著更高。抗 TOPO 在所有患者中的检出率为 71%，在 dcSSC 中为 90.5%，在 lcSSc 中为 65.8%。抗 TOPO 与 dcSSc、更高的皮肤评分、手指溃疡/坏疽、肺纤维化、DLCO<70%显著相关。U1-RNP 抗体与肺纤维化程度较低相关。ACA 阳性率为 7%，仅存在于 lcSSc 患者中。我们没有发现性别与自身抗体存在之间的关联。抗 TOPO 抗体的患病率较高，而 ACA 抗体的患病率较低。在抗 TOPO 和 ACA 阳性的患者中，疾病的临床亚型没有差异。自身抗体的患病率差异提示需要进一步进行遗传研究。
