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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, MC. Vonk, A. Garaiman, BE. Kersten, R. Becvar, M. Tomcik, AM. Hoffmann-Vold, I. Castellvi, JT. Jaime, M. Brzosko, M. Milchert, D. Krasowska, M. Michalska-Jakubus, P. Airo, M. Matucci-Cerinic, C. Bruni, M. Iudici, J. Distler, AM....

. 2024 ; 125 (-) : 104-110. [pub] 20240409

Jazyk angličtina Země Nizozemsko

Typ dokumentu časopisecké články, systematický přehled

Perzistentní odkaz   https://www.medvik.cz/link/bmc24019931

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. OBJECTIVES: 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 %. CONCLUSION: 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 %.

Department of Biomedical Sciences Humanitas University via R Levi Montalcini 20090 Pieve Emanuele Italy

Department of Dermatology Venereology and Pediatric Dermatology Medical University of Lublin Staszica 11L 20 081 Lublin Poland

Department of Experimental and Clinical Medicine University of Florence and Division of Rheumatology AOUC Florence Italy

Department of Internal Medicine and Rheumatology Clinic Ion Cantacuzino Hospital Ion Movila Street 5 7 020475 Bucharest Romania

Department of Internal Medicine Rheumatology Diabetology Geriatrics and Clinical Immunology Pomeranian Medical University in Szczecin Ul Unii Lubelskiej 1 71 252 Szczecin Poland

Department of Rheumatology Colentina Clinical Hospital 19 21 Stefan cel Mare 020125 Bucharest Romania

Department of Rheumatology Firoozgar Hospital Beh Afarin street Tehran Iran

Department of Rheumatology Hospital Universitari de la Santa Creu i Sant Pau Sant Antoni Maria Claret 167 Barcelona Spain

Department of Rheumatology Rikshospitalet University Hospital Sognsvannveien 20 0027 Oslo Norway

Department of Rheumatology University Hospital Düsseldorf Heinrich Heine University Düsseldorf Germany

Department of Rheumatology University Hospital Zurich University of Zurich Rämistrasse 100 CH 8091 Zürich Switzerland

Department of the Rheumatology Radboud University Nijmegen Medical Centre Huispost 667 6500HB Nijmegen the Netherlands

Institute of Rheumatology Department of Rheumatology 1st Faculty of Medicine Charles University Na Slupi 4 12800 Praha 2 Czechia

Rheumatology and Clinical Immunology IRCCS Humanitas Research Hospital via A Manzoni 56 20089 Rozzano Milan Italy

Rheumatology Unit Geneva University Hospitals 1211 Geneva 14 Switzerland

Spedali Civili di Brescia Scleroderma UNIT UOC Reumatologia ed Immunologia Clinica Piazzale Spedali Civili 1 25123 Brescia Italy

Unit of Immunology Rheumatology Allergy and Rare Diseases IRCCS San Raffaele Scientific Institute Vita Salute San Raffaele University Milan Italy

Citace poskytuje Crossref.org

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$a 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. OBJECTIVES: 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 %. CONCLUSION: 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 %.
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