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The aim of the present multicentric study was to determine the clinical relevance of antifibrillarin autoantibodies (AFA<\/jats:styled-content>) in patients with SS<\/jats:styled-content>c. The clinical features of 37 patients with SS<\/jats:styled-content>c positive for AFA<\/jats:styled-content> (AFA<\/jats:styled-content>+) and 139 SS<\/jats:styled-content>c patients without AFA<\/jats:styled-content> (AFA<\/jats:styled-content>\u2212) were collected retrospectively from medical records to enable a comparison between AFA<\/jats:styled-content>\u2212 and AFA<\/jats:styled-content>+ patients. Antifibrillarin autoantibodies were screened by an indirect immunofluorescence technique using HE<\/jats:styled-content>p2 cells and identified by an in\u2010house Western blot technique and\/or an EliA test. Comparing AFA<\/jats:styled-content>+ and AFA<\/jats:styled-content>\u2212 patients, AFA<\/jats:styled-content>+ patients were significantly younger at disease onset (36.9 versus 42.9; P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.02), more frequently male (P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.02) and of Afro\u2010Caribbean descent (65% versus 7.7%; P\u00a0<\/jats:italic><\u00a0<\/jats:italic>0.001). At diagnosis, the Rodnan skin score evaluating the cutaneous manifestations was higher (13.3 versus 8.7; P\u00a0<\/jats:italic>=\u00a0<\/jats:italic>0.01) and myositis was also more common in the AFA<\/jats:styled-content>+ group (31.4% versus 12.2%; P<\/jats:italic>\u00a0<\u00a00.01). Patients with AFA<\/jats:styled-content>+ were not associated with diffuse cutaneous SS<\/jats:styled-content>c or with lung involvement and no difference in survival was observed. Antifibrillarin autoantibodies are associated with patients of Afro\u2010Caribbean origin and can identify patients with SS<\/jats:styled-content>c who are younger at disease onset and display a higher prevalence of myositis.<\/jats:p>","DOI":"10.1111\/sji.12510","type":"journal-article","created":{"date-parts":[[2016,11,16]],"date-time":"2016-11-16T20:02:15Z","timestamp":1479326535000},"page":"73-79","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":34,"title":["The Clinical Relevance of Antifibrillarin (anti\u2010U3\u2010RNP<\/scp>) Autoantibodies in Systemic Sclerosis"],"prefix":"10.1111","volume":"85","author":[{"given":"F.","family":"Tall","sequence":"first","affiliation":[{"name":"Immunology Department AP\u2010HP Saint\u2010Antoine Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Dechomet","sequence":"additional","affiliation":[{"name":"Immunology Department Lyon\u2010Sud Hospital Hospices Civils de Lyon; Claude Bernard Pierre\u2010Benite France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Riviere","sequence":"additional","affiliation":[{"name":"Internal Medicine Department AP\u2010HP Saint\u2010Antoine Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.","family":"Cottin","sequence":"additional","affiliation":[{"name":"Respiratory Diseases Department Louis Pradel Hospital Bron France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Ballot","sequence":"additional","affiliation":[{"name":"Immunology Department AP\u2010HP Saint\u2010Antoine Hospital Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K. 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