%0 Journal Article
%T Clinical significance of the anti-Nucleolar Organizer Region 90 antibodies (NOR90) in systemic sclerosis: Analysis of the European Scleroderma Trials and Research (EUSTAR) cohort and a systematic literature review.
%A Dima A
%A Vonk MC
%A Garaiman A
%A Kersten BE
%A Becvar R
%A Tomcik M
%A Hoffmann-Vold AM
%A Castellvi I
%A Jaime JT
%A Brzosko M
%A Milchert M
%A Krasowska D
%A Michalska-Jakubus M
%A Airo P
%A Matucci-Cerinic M
%A Bruni C
%A Iudici M
%A Distler J
%A Gheorghiu AM
%A Poormoghim H
%A Motta F
%A De Santis M
%A Parvu M
%A Distler O
%A Mihai C
%J Eur J Intern Med
%V 125
%N 0
%D 2024 Jul 9
%M 38599922
%F 7.749
%R 10.1016/j.ejim.2024.03.035
%X <B>BACKGROUND: </B>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.<BR><B>OBJECTIVE: </B>To analyze the clinical associations of NOR90 in patients with SSc in a multicentric cohort.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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 %.<BR><B>CONCLUSIONS: </B>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 %.