{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T06:35:56Z","timestamp":1781850956390,"version":"3.54.5"},"reference-count":10,"publisher":"The Journal of Rheumatology","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2023,8]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Systemic sclerosis (SSc) is burdened by Raynaud phenomenon (RP) and digital ulcers (DUs), and sometimes standard vasoactive therapies are ineffective or contraindicated. Selexipag is an oral selective IP prostacyclin receptor agonist approved for the treatment of SSc-related pulmonary arterial hypertension. We aimed to evaluate the clinical and instrumental efficacy of selexipag in SSc digital vasculopathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies were administered selexipag. RP- and DU-related clinical outcomes were evaluated, and digital perfusion was assessed by laser speckle contrast analysis (LASCA), all at baseline and after 3 months.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Selexipag was administered to 9 patients with SSc (66.6% female, mean age 52.3 [SD 16.6] yrs). One patient had to stop the drug because of adverse effects. After 3 months of selexipag administration, there was a significant reduction in RP daily episodes (<jats:italic>P<\/jats:italic>= 0.01) and RP mean duration (<jats:italic>P<\/jats:italic>= 0.04). The number of DUs decreased from 10 to 4 without reaching statistical significance. A significant improvement in mean perfusion of the fingers (<jats:italic>P<\/jats:italic>= 0.02) was observed with LASCA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Selexipag showed good potential for the treatment of SSc digital vasculopathy. Our results are certainly preliminary, yet quite encouraging. New trials for the evaluation of selexipag efficacy in SSc digital vasculopathy are needed.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.221113","type":"journal-article","created":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T12:20:23Z","timestamp":1680351623000},"page":"1029-1031","source":"Crossref","is-referenced-by-count":6,"title":["Preliminary Clinical and Laser Speckle Contrast Analysis Data on Selexipag Efficacy for the Treatment of Digital Vasculopathy in Systemic Sclerosis"],"prefix":"10.3899","volume":"50","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4788-5729","authenticated-orcid":false,"given":"Marco","family":"Di Battista","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Della Rossa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mattia","family":"Da Rio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giammarco","family":"De Mattia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo","family":"Morganti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5937-4574","authenticated-orcid":false,"given":"Marta","family":"Mosca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2023,4,1]]},"reference":[{"key":"2023122105350779000_50.8.1029.1","doi-asserted-by":"publisher","DOI":"10.1002\/art.37988"},{"key":"2023122105350779000_50.8.1029.2","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209909"},{"key":"2023122105350779000_50.8.1029.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1503184"},{"key":"2023122105350779000_50.8.1029.4","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02493-2016"},{"key":"2023122105350779000_50.8.1029.5","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"2023122105350779000_50.8.1029.6","doi-asserted-by":"crossref","first-page":"104307","DOI":"10.1016\/j.mvr.2021.104307","article-title":"Definition and application of proximal-distal gradient finger perfusion in systemic sclerosis by laser speckle contrast analysis","volume":"140","author":"Di Battista","year":"2022","journal-title":"Microvasc Res"},{"key":"2023122105350779000_50.8.1029.7","doi-asserted-by":"crossref","first-page":"686","DOI":"10.1016\/j.semarthrit.2018.03.019","article-title":"Practical suggestions on intravenous iloprost in Raynaud\u2019s phenomenon and digital ulcer secondary to systemic sclerosis: systematic literature review and expert consensus","volume":"48","author":"Ingegnoli","year":"2019","journal-title":"Semin Arthritis Rheum"},{"key":"2023122105350779000_50.8.1029.8","first-page":"181","article-title":"Selexipag may be effective in inducing digital ulcers healing in patients with systemic sclerosis","volume":"38","author":"Del Papa","year":"2020","journal-title":"Clin Exp Rheumatol"},{"key":"2023122105350779000_50.8.1029.9","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.200716"},{"key":"2023122105350779000_50.8.1029.10","doi-asserted-by":"crossref","first-page":"2370","DOI":"10.1002\/art.40242","article-title":"Efficacy and safety of selexipag in adults with Raynaud\u2019s phenomenon secondary to systemic sclerosis: a randomized, placebo-controlled, phase II study","volume":"69","author":"Denton","year":"2017","journal-title":"Arthritis Rheumatol"}],"container-title":["The Journal of Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.3899\/jrheum.221113","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,21]],"date-time":"2023-12-21T13:35:39Z","timestamp":1703165739000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.jrheum.org\/lookup\/doi\/10.3899\/jrheum.221113"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,4,1]]},"references-count":10,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2023,8,1]]},"published-print":{"date-parts":[[2023,8]]}},"alternative-id":["10.3899\/jrheum.221113"],"URL":"https:\/\/doi.org\/10.3899\/jrheum.221113","relation":{},"ISSN":["0315-162X","1499-2752"],"issn-type":[{"value":"0315-162X","type":"print"},{"value":"1499-2752","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,4,1]]}}}