Terras Sarah, Hartenstein Hans, Höxtermann Stefan, Gambichler Thilo, Kreuter Alexander
Department of Dermatology, Venereology, and Allergology, Ruhr-University Bochum, Bochum, Germany.
Department of Dermatology, Venereology, and Allergology, HELIOS St. Elisabeth Hospital, Oberhausen, Germany.
Int J Dermatol. 2016 Aug;55(8):882-5. doi: 10.1111/ijd.13032. Epub 2015 Oct 24.
Systemic sclerosis (SSc) is a multiorgan autoimmune disorder characterized by sclerosis of the skin and organs as well as the presence of antinuclear autoantibodies. Several types of antinuclear autoantibodies have been described in SSc, associated with distinct disease entities and differences in prognosis.
The aim of this study was to screen for the presence of antibodies reacting with RNA polymerase III (anti-RNAP3) in a large cohort of patients with SSc treated at a tertiary referral center and to evaluate correlations with disease severity.
Anti-RNAP3 antibodies were detected in 11 of 158 patients (7.0%). Eight of the 11 (72.7%) anti-RNAP3-positive patients had diffuse cutaneous SSc (P < 0.01). A higher modified Rodnan skin score, associated with diffuse SSc, correlated with the presence of anti-RNAP3 (P < 0.0001). The detection of anti-RNAP3 antibodies strongly correlated with the presence of renal involvement (P < 0.0001). The odds ratio of RNAP3-positive patients to develop renal involvement was 80.1 (95% CI 9.3-690.1).
This study demonstrates that the detection of anti-RNAP3 antibodies in patients with SSc correlates with renal crisis and severe cutaneous involvement. The possibility to detect specific antibodies with a prognostic value can lead to a better risk management of patients with SSc.
系统性硬化症（SSc）是一种多器官自身免疫性疾病，其特征为皮肤和器官硬化以及存在抗核自身抗体。在SSc中已描述了几种类型的抗核自身抗体，它们与不同的疾病实体及预后差异相关。
本研究的目的是在一家三级转诊中心接受治疗的一大群SSc患者中筛查与RNA聚合酶III反应的抗体（抗RNAP3）的存在情况，并评估其与疾病严重程度的相关性。
158例患者中有11例（7.0%）检测到抗RNAP3抗体。11例抗RNAP3阳性患者中有8例（72.7%）患有弥漫性皮肤型SSc（P < 0.01）。与弥漫性SSc相关的改良Rodnan皮肤评分越高，与抗RNAP3的存在相关（P < 0.0001）。抗RNAP3抗体的检测与肾脏受累的存在密切相关（P < 0.0001）。RNAP3阳性患者发生肾脏受累的比值比为80.1（95%可信区间9.3 - 690.1）。
本研究表明，在SSc患者中检测抗RNAP3抗体与肾脏危象和严重皮肤受累相关。检测具有预后价值的特异性抗体的可能性可导致对SSc患者进行更好的风险管理。
