Abstract：
BACKGROUND: The anti-Nucleolar Organizer Region 90 antibodies (NOR90) are rare antinuclear antibodies (ANA) reported in systemic sclerosis (SSc). Especially due to low prevalence, the clinical relevance of NOR90 in SSc remains uncertain.
OBJECTIVE: To analyze the clinical associations of NOR90 in patients with SSc in a multicentric cohort.
METHODS: Post-hoc, cross-sectional study of prospectively collected data from the European Scleroderma Trials and Research (EUSTAR) database, with additional information on NOR90. Further, we performed a systematic literature search, using the terms \"systemic sclerosis\" and \"NOR90\" across three databases: Medline via PubMed, Scopus, and Thomson Reuters\' Web of Science Core Collection, from inception to November 1st, 2023.
RESULTS: Overall, 1318 patients with SSc were included (mean age 58.3 ± 13.7 years, 81.3 % female), of whom 44 (3.3 %) were positive for NOR90. Of these, 32 were also positive for one of the SSc-criteria antibodies: 9/44 (20.5 %) for anti-topoisomerase I, 18/42 (42.9 %) for anti-centromere, and 5/40 (12.5 %) for anti-RNA polymerase III. NOR90-positive patients were more frequently female, had lower modified Rodnan skin score (mRSS), and lower prevalence of upper and lower gastrointestinal (GI) symptoms compared to NOR90-negative patients. In multivariable analysis, NOR90 remained significantly associated with lower mRSS and less frequent GI symptoms. The literature search identified 17 articles, including a total number of 87 NOR90-positive out of 3357 SSc patients, corresponding to an overall prevalence of 2.6 %.
CONCLUSIONS: To our best knowledge, this is the largest SSc cohort tested for NOR90 to date, confirming the NOR90 prevalence in SSc patients is around 3 %.
OBJECTIVE: To analyze the clinical associations of NOR90 in patients with SSc in a multicentric cohort.
METHODS: Post-hoc, cross-sectional study of prospectively collected data from the European Scleroderma Trials and Research (EUSTAR) database, with additional information on NOR90. Further, we performed a systematic literature search, using the terms \"systemic sclerosis\" and \"NOR90\" across three databases: Medline via PubMed, Scopus, and Thomson Reuters\' Web of Science Core Collection, from inception to November 1st, 2023.
RESULTS: Overall, 1318 patients with SSc were included (mean age 58.3 ± 13.7 years, 81.3 % female), of whom 44 (3.3 %) were positive for NOR90. Of these, 32 were also positive for one of the SSc-criteria antibodies: 9/44 (20.5 %) for anti-topoisomerase I, 18/42 (42.9 %) for anti-centromere, and 5/40 (12.5 %) for anti-RNA polymerase III. NOR90-positive patients were more frequently female, had lower modified Rodnan skin score (mRSS), and lower prevalence of upper and lower gastrointestinal (GI) symptoms compared to NOR90-negative patients. In multivariable analysis, NOR90 remained significantly associated with lower mRSS and less frequent GI symptoms. The literature search identified 17 articles, including a total number of 87 NOR90-positive out of 3357 SSc patients, corresponding to an overall prevalence of 2.6 %.
CONCLUSIONS: To our best knowledge, this is the largest SSc cohort tested for NOR90 to date, confirming the NOR90 prevalence in SSc patients is around 3 %.
摘要：
背景：抗核仁组织区90抗体（NOR90）是系统性硬化症（SSc）中报道的罕见抗核抗体（ANA）。特别是由于患病率低，NOR90在SSc中的临床意义仍不确定.
目的：分析多中心队列中SSc患者NOR90的临床相关性。
方法：事后，从欧洲硬皮病试验和研究（EUSTAR）数据库中前瞻性收集的数据的横断面研究，有关NOR90的其他信息。Further,我们进行了系统的文献检索，在三个数据库中使用术语“系统性硬化症”和“NOR90”：MedlineviaPubMed，Scopus,和汤森路透的科学核心合集，从成立到11月1日，2023年。
结果：总体而言，纳入1318例SSc患者(平均年龄58.3±13.7岁，81.3%女性)，其中44人（3.3%）NOR90阳性。其中,32对SSc标准抗体之一也为阳性：抗拓扑异构酶I的9/44（20.5％），18/42（42.9%）用于抗着丝粒，和5/40（12.5％）的抗RNA聚合酶III。NOR90阳性患者更常见的是女性，具有较低的改良Rodnan皮肤评分（mRSS），与NOR90阴性患者相比，上消化道和下消化道（GI）症状的患病率较低。在多变量分析中，NOR90仍然与较低的mRSS和较不频繁的胃肠道症状显著相关。文献检索确定了17篇文章，包括3357名SSc患者中的87名NOR90阳性患者，相当于2.6%的总体患病率。
结论：据我们所知，这是迄今为止测试的NOR90最大的SSc队列，证实SSc患者的NOR90患病率约为3%。
目的：分析多中心队列中SSc患者NOR90的临床相关性。
方法：事后，从欧洲硬皮病试验和研究（EUSTAR）数据库中前瞻性收集的数据的横断面研究，有关NOR90的其他信息。Further,我们进行了系统的文献检索，在三个数据库中使用术语“系统性硬化症”和“NOR90”：MedlineviaPubMed，Scopus,和汤森路透的科学核心合集，从成立到11月1日，2023年。
结果：总体而言，纳入1318例SSc患者(平均年龄58.3±13.7岁，81.3%女性)，其中44人（3.3%）NOR90阳性。其中,32对SSc标准抗体之一也为阳性：抗拓扑异构酶I的9/44（20.5％），18/42（42.9%）用于抗着丝粒，和5/40（12.5％）的抗RNA聚合酶III。NOR90阳性患者更常见的是女性，具有较低的改良Rodnan皮肤评分（mRSS），与NOR90阴性患者相比，上消化道和下消化道（GI）症状的患病率较低。在多变量分析中，NOR90仍然与较低的mRSS和较不频繁的胃肠道症状显著相关。文献检索确定了17篇文章，包括3357名SSc患者中的87名NOR90阳性患者，相当于2.6%的总体患病率。
结论：据我们所知，这是迄今为止测试的NOR90最大的SSc队列，证实SSc患者的NOR90患病率约为3%。
