{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,2]],"date-time":"2026-09-02T18:21:25Z","timestamp":1788373285679,"version":"build-2803163510"},"reference-count":26,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T00:00:00Z","timestamp":1778630400000},"content-version":"unspecified","delay-in-days":42,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2026,3,23]]},"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Antitopoisomerase I (ATA), anticentromere (ACA) and anti-RNA polymerase III (RNAP3) antibodies are included in the 2013 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for systemic sclerosis (SSc). A subset of patients with SSc satisfy criteria but may lack these specific autoantibodies, being classified as \u2018triple-negative\u2019.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a retrospective evaluation of triple-negative patients with SSc prevalence and clinical features among the multicentric Systemic sclerosis Progression INvestiGation registry.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Out of 1480 patients with SSc, 295 (19.9%) were triple-negative, while 1185 (81.1%) had SSc-specific antibodies: ACA (54.3%), ATA (43.6%) and RNAP3 (2.1%). The triple-negative group showed a higher prevalence of myopathy (16.7% vs 10.1%, p=0.003), suggested by higher creatine phosphokinase (CPK) levels (126.2 vs 92.5 U\/mL, p=0.002), more frequent CPK increase over 2\u20133\u2009times (2.4% vs 0.2%, p=0.028). Triple-negative patients also exhibited fewer vascular complications, including digital ulcers (17.3% vs 22.8%, p=0.04) and calcinosis (8.2% vs 12.8%, p=0.027), and a higher prevalence of interstitial lung disease (p&lt;0.001). Consistently, lower diffusing capacity for carbon monoxide (66.4% vs 70.98%, p=0.004) and forced vital capacity (97.01% vs 102.92%, p&lt;0.001) were found in the triple-negative group. Triple-negative patients more frequently received corticosteroids (79.3% vs 67.9%, p=0.003), cyclophosphamide (43.4% vs 26%, p&lt;0.001) and azathioprine (38.5% vs 22.3%, p=0.002), while less frequently received prostanoids (71.6% vs 85.9%, p&lt;0.001), calcium channel blockers (80.1% vs 87.7%, p=0.005) and phosphodiesterase-5 inhibitors (4% vs 20%, p&lt;0.001).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>A higher prevalence of myopathy and interstitial lung disease and a reduced vascular burden were found in the triple-negative patients, suggesting that the non-specific and non-routinely tested autoantibodies may identify an SSc endotype resembling sclero-myositis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/rmdopen-2025-006495","type":"journal-article","created":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T14:26:05Z","timestamp":1778682365000},"page":"e006495","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical and serological features of triple autoantibody-negative patients with systemic sclerosis: insights from the multicentric SPRING registry of the Italian Society for Rheumatology"],"prefix":"10.1136","volume":"12","author":[{"given":"Veronica","family":"Batani","sequence":"first","affiliation":[{"name":"Vita-Salute San Raffaele University, Milan, Italy"},{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Inflammation, Fibrosis and Ageing Initiative (INFLAGE), IRCCS Ospedale San Raffaele, Milan, Italy"},{"name":"Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Cavazzana","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, Brescia, Italy"},{"name":"Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Orlandi","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, School of Medicine, University of Modena and Reggio Emilia, Modena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5169-3511","authenticated-orcid":false,"given":"Rossella","family":"De Angelis","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Ancona, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6806-3794","authenticated-orcid":false,"given":"Corrado","family":"Campochiaro","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Inflammation, Fibrosis and Ageing Initiative (INFLAGE), IRCCS Ospedale San Raffaele, Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9819-105X","authenticated-orcid":false,"given":"Enrico","family":"De Lorenzis","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7333-371X","authenticated-orcid":false,"given":"Gerlando","family":"Natalello","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucrezia","family":"Verardi","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. 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