Laboratory Medicine, Immunology, University Hospitals Leuven, Leuven, Belgium.
Clin Chim Acta. 2010 Jul 4;411(13-14):965-71. doi: 10.1016/j.cca.2010.03.018. Epub 2010 Mar 25.
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by the presence of various autoantibodies, including anti-centromere, anti-topoisomerase (Scl-70), anti-PM/Scl-100, and anti-RNA-polymerase III (RNA Pol-III) antibodies. Recently, new ELISA based immunoassays have become available for the detection of anti-PM/Scl and anti-RNA Pol-lII antibodies.
We studied the prevalence and clinical association of anti-PM/Scl-100 (PM1-Alpha) and anti-RNA Pol-III antibodies.
Antibodies to PM1-Alpha and RNA Pol-III were measured by ELISA (DR. Fooke Laboratories and Inova Diagnostics, respectively) in 242 patients with various connective tissue diseases (CTD) (including 70 SSc patients) and in 36 non-CTD controls.
Low levels of PM1-Alpha antibodies were found in various CTDs, whereas high levels were exclusively found in SSc, dermatomyositis and polymyositis, albeit at low frequency (4.7%). Anti-RNA Pol-III antibodies were found in 7% of SSc and in 1% of non-CTD and CTD controls. Anti-centromere and anti-Scl-70 antibodies were found in 37% and 21% of SSc patients, respectively. Anti-centromere antibodies were associated with limited cutaneous SSc and anti-Scl-70 antibodies with diffuse cutaneous SSc and interstitial lung disease. Because of the low number of samples positive for anti-PM/Scl-100 or RNA Pol-III antibodies, no clinical feature was statistically correlated with the presence of either reactivity, but taken together the presence of either antibody was correlated with interstitial lung disease. Anti-PM1-Alpha and anti-RNA Pol-III antibodies were mutually exclusive with anti-Scl-70 antibodies.
At high levels, anti-PM/Scl-100 antibodies were associated with SSc, PM, and DM, albeit at low frequency. Anti-RNA Pol-III antibodies were associated with SSc (in 7%) with high specificity.
系统性硬化症（SSc）是一种罕见的自身免疫性疾病，其特征是存在各种自身抗体，包括抗着丝点、抗拓扑异构酶（Scl-70）、抗-PM/Scl-100 和抗 RNA 聚合酶 III（RNA Pol-III）抗体。最近，新的基于 ELISA 的免疫测定法已可用于检测抗-PM/Scl 和抗 RNA Pol-lII 抗体。
我们研究了抗-PM/Scl-100（PM1-Alpha）和抗 RNA Pol-III 抗体的流行率和临床关联。
采用 ELISA（分别由 DR. Fooke Laboratories 和 Inova Diagnostics 生产）检测 242 例各种结缔组织疾病（CTD）患者（包括 70 例 SSc 患者）和 36 名非 CTD 对照者的 PM1-Alpha 和 RNA Pol-III 抗体。
在各种 CTD 中发现了低水平的 PM1-Alpha 抗体，而高水平的 PM1-Alpha 抗体仅在 SSc、皮肌炎和多发性肌炎中发现，尽管频率较低（4.7%）。抗 RNA Pol-III 抗体在 7%的 SSc 患者和 1%的非 CTD 和 CTD 对照者中发现。抗着丝点和抗 Scl-70 抗体在 37%和 21%的 SSc 患者中发现。抗着丝点抗体与局限性皮肤 SSc 相关，抗 Scl-70 抗体与弥漫性皮肤 SSc 和间质性肺病相关。由于抗 PM/Scl-100 或 RNA Pol-III 抗体阳性的样本数量较少，因此没有临床特征与任何一种反应性呈统计学相关，但总的来说，任何一种抗体的存在都与间质性肺病相关。抗 PM1-Alpha 和抗 RNA Pol-III 抗体与抗 Scl-70 抗体相互排斥。
高水平的抗-PM/Scl-100 抗体与 SSc、PM 和 DM 相关，尽管频率较低。抗 RNA Pol-III 抗体与 SSc（7%）相关，特异性高。
