• A Dima, M C Vonk, A Garaiman, B E Kersten, R Becvar, M Tomcik, A-M Hoffmann-Vold, I Castellvi, Jl Tandaipan Jaime, M Brzosko, M Milchert, D Krasowska, M Michalska-Jakubus, P Airo, M Matucci-Cerinic, C Bruni, M Iudici, Jhw Distler, A M Gheorghiu, H Poormoghim, F Motta, M De Santis, M Parvu, O Distler, and C Mihai.
• Department of Rheumatology, University Hospital Zurich, University of Zurich, Rämistrasse 100, CH-8091 Zürich, Switzerland; Department of Rheumatology, Colentina Clinical Hospital, 19-21 Stefan cel Mare, 020125 Bucharest, Romania. Electronic address: alina_dima@outlook.com.
• Eur. J. Intern. Med. 2024 Jul 1; 125: 104110104-110.

BackgroundThe 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.ObjectivesTo analyze the clinical associations of NOR90 in patients with SSc in a multicentric cohort.MethodsPost-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.ResultsOverall, 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 %.ConclusionTo our best knowledge, this is the largest SSc cohort tested for NOR90 to date, confirming the NOR90 prevalence in SSc patients is around 3 %.Copyright © 2024 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.

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