{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,2]],"date-time":"2026-11-02T08:52:46Z","timestamp":1793609566450,"version":"4.5.0"},"reference-count":58,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2023,3,27]],"date-time":"2023-03-27T00:00:00Z","timestamp":1679875200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Anti-nuclear (ANA) are present in approximately 90% of systemic sclerosis (SSc) patients and are key biomarkers in supporting the diagnosis and determining the prognosis of this disease. In addition to the classification criteria autoantibodies for SSc [i.e., anti-centromere, anti-topoisomerase I (Scl-70), anti-RNA polymerase III], other autoantibodies have been associated with important SSc phenotypes. Among them, anti-U11\/U12 ribonucleoprotein (RNP) antibodies, also known as anti-RNPC-3, were first reported in a patient with SSc, but very little is known about their association and clinical utility. The U11\/U12 RNP macromolecular complex consists of several proteins involved in alternative mRNA splicing. More recent studies demonstrated associations of anti-anti-U11\/U12 antibodies with SSc and severe pulmonary fibrosis as well as with moderate to severe gastrointestinal dysmotility. Lastly, anti-U11\/U12 autoantibodies have been strongly associated with malignancy in SSc patients. Here, we aimed to summarize the knowledge of anti-U11\/U12\/RNPC-3 antibodies in SSc, including their seroclinical associations in a narrative literature review.<\/jats:p>","DOI":"10.3390\/diagnostics13071257","type":"journal-article","created":{"date-parts":[[2023,3,27]],"date-time":"2023-03-27T07:32:09Z","timestamp":1679902329000},"page":"1257","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Anti-U11\/U12 Antibodies as a Rare but Important Biomarker in Patients with Systemic Sclerosis: A Narrative Review"],"prefix":"10.3390","volume":"13","author":[{"given":"Marvin J.","family":"Fritzler","sequence":"first","affiliation":[{"name":"Cumming School of Medicine, University of Calgary, Calgary, AB T2N 4N1, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chelsea","family":"Bentow","sequence":"additional","affiliation":[{"name":"Research and Development, Werfen, Autoimmunity Headquarters and Technology Center, San Diego, CA 92131-1638, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6529-6258","authenticated-orcid":false,"given":"Lorenzo","family":"Beretta","sequence":"additional","affiliation":[{"name":"Scleroderma Unit and (Referral) Center for Systemic Autoimmune Diseases, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico di Milan, 20122 Milano, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2499-8250","authenticated-orcid":false,"given":"Boaz","family":"Palterer","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, University of Florence, 50121 Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janire","family":"Perurena-Prieto","sequence":"additional","affiliation":[{"name":"Department of Immunology, Hospital Universitari Vall d\u2019Hebron, Universitat Aut\u00f2noma de Barcelona, 08193 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5814-8698","authenticated-orcid":false,"given":"Maria Teresa","family":"Sanz-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Department of Immunology, Hospital Universitari Vall d\u2019Hebron, Universitat Aut\u00f2noma de Barcelona, 08193 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0626-507X","authenticated-orcid":false,"given":"Alfredo","family":"Guillen-Del-Castillo","sequence":"additional","affiliation":[{"name":"Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d\u2019Hebron, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2873-456X","authenticated-orcid":false,"given":"Ana","family":"Mar\u00edn","sequence":"additional","affiliation":[{"name":"Department of Immunology, Hospital Universitari Vall d\u2019Hebron, Universitat Aut\u00f2noma de Barcelona, 08193 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicent","family":"Fonollosa-Pla","sequence":"additional","affiliation":[{"name":"Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d\u2019Hebron, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Callejas-Moraga","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital P\u00fablico de Monforte, 27400 Lugo, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3390-9029","authenticated-orcid":false,"given":"Carmen Pilar","family":"Sime\u00f3n-Aznar","sequence":"additional","affiliation":[{"name":"Unit of Systemic Autoimmune Diseases, Department of Internal Medicine, Hospital Universitari Vall d\u2019Hebron, 08035 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Mahler","sequence":"additional","affiliation":[{"name":"Research and Development, Werfen, Autoimmunity Headquarters and Technology Center, San Diego, CA 92131-1638, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"102583","DOI":"10.1016\/j.autrev.2020.102583","article-title":"Autoantibodies to stratify systemic sclerosis patients into clinically actionable subsets","volume":"19","author":"Mahler","year":"2020","journal-title":"Autoimmun. 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