{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T06:35:49Z","timestamp":1781850949332,"version":"3.54.5"},"reference-count":18,"publisher":"The Journal of Rheumatology","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"accepted":{"date-parts":[[2024,6,4]]},"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Raynaud phenomenon (RP) and digital ulcers (DUs) are the main signs of digital vasculopathy in systemic sclerosis (SSc). Selexipag is an oral prostacyclin agonist approved for SSc-related pulmonary arterial hypertension. Following our previous preliminary short-course report, we herein present long-term data on selexipag safety and efficacy in the treatment of SSc digital vasculopathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Selexipag was administered to patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies. Each subject was assessed at baseline and after 3, 6, and 12 months. Clinical outcomes related to RP and DUs were evaluated along with modified Rodnan skin score of the fingers. Digital perfusion was assessed by laser speckle contrast analysis (LASCA). Nailfold videocapillaroscopy (NVC) was also performed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eight patients with SSc (63% female, mean age 50.1 years) received selexipag. After 12 months of treatment, RP was reported to significantly decrease in the number of daily episodes and mean duration (<jats:italic>P<\/jats:italic>&lt; 0.001 and<jats:italic>P<\/jats:italic>= 0.01, respectively). All patients achieved a complete healing of their DUs (<jats:italic>P<\/jats:italic>= 0.03) within 6 months. A progressive reduction of fingers skin score was observed (<jats:italic>P<\/jats:italic>= 0.03). No structural changes of capillaries were noted on NVC. Conversely, LASCA revealed an important increase in total digital perfusion (<jats:italic>P<\/jats:italic>= 0.004) despite seasonal variability. The safety profile was consistent with that reported in the literature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We observed a sustained efficacy of selexipag on SSc digital vasculopathy during 1 year of administration. Our promising results encourage the design of a new randomized controlled trial to evaluate the effect of selexipag on SSc digital vasculopathy.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.2024-0103","type":"journal-article","created":{"date-parts":[[2024,6,15]],"date-time":"2024-06-15T12:20:27Z","timestamp":1718454027000},"page":"899-903","source":"Crossref","is-referenced-by-count":6,"title":["Long-Term Data on Efficacy and Safety of Selexipag for Digital Systemic Sclerosis Vasculopathy"],"prefix":"10.3899","volume":"51","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"}]},{"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":[[2024,6,15]]},"reference":[{"key":"2024090105300581000_51.9.899.1","doi-asserted-by":"crossref","first-page":"208","DOI":"10.1038\/s41584-020-0386-4","article-title":"Raynaud phenomenon and digital ulcers in systemic sclerosis","volume":"16","author":"Hughes","year":"2020","journal-title":"Nat Rev Rheumatol"},{"key":"2024090105300581000_51.9.899.2","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209909"},{"key":"2024090105300581000_51.9.899.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1503184"},{"key":"2024090105300581000_51.9.899.4","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02493-2016"},{"key":"2024090105300581000_51.9.899.5","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.221113"},{"key":"2024090105300581000_51.9.899.6","doi-asserted-by":"crossref","first-page":"11","DOI":"10.5301\/jsrd.5000231","article-title":"Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis","volume":"2","author":"Khanna","year":"2017","journal-title":"J Scleroderma Relat Disord"},{"key":"2024090105300581000_51.9.899.7","first-page":"155","article-title":"Nailfold videocapillaroscopy assessment of microvascular damage in systemic sclerosis","volume":"27","author":"Cutolo","year":"2000","journal-title":"J Rheumatol"},{"key":"2024090105300581000_51.9.899.8","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":"2024090105300581000_51.9.899.9","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":"2024090105300581000_51.9.899.10","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"},{"key":"2024090105300581000_51.9.899.11","first-page":"503","article-title":"Effects of long-term cyclic iloprost therapy in systemic sclerosis with Raynaud\u2019s phenomenon. A randomized, controlled study","volume":"19","author":"Scorza","year":"2001","journal-title":"Clin Exp Rheumatol"},{"key":"2024090105300581000_51.9.899.12","doi-asserted-by":"publisher","DOI":"10.1007\/PL00011223"},{"key":"2024090105300581000_51.9.899.13","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.081044"},{"key":"2024090105300581000_51.9.899.14","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1186\/s42358-019-0057-9","article-title":"Risk of digital ulcers occurrence in systemic sclerosis: a cross-sectional study","volume":"59","author":"Coelho Horimoto","year":"2019","journal-title":"Adv Rheumatol"},{"key":"2024090105300581000_51.9.899.15","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1007\/s12325-021-01898-1","article-title":"Long-term survival, safety and tolerability with selexipag in patients with pulmonary arterial hypertension: results from GRIPHON and its open-label extension","volume":"39","author":"Gali\u00e8","year":"2022","journal-title":"Adv Ther"},{"key":"2024090105300581000_51.9.899.16","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1016\/j.healun.2023.09.016","article-title":"Disease characteristics, treatments, and outcomes of patients with pulmonary arterial hypertension treated with selexipag in real-world settings from the SPHERE registry (SelexiPag: tHe usErs dRug rEgistry)","volume":"43","author":"McLaughlin","year":"2024","journal-title":"J Heart Lung Transplant"},{"key":"2024090105300581000_51.9.899.17","doi-asserted-by":"publisher","DOI":"10.1002\/art.10486"},{"key":"2024090105300581000_51.9.899.18","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1016\/j.autrev.2018.01.023","article-title":"Is laser speckle contrast analysis (LASCA) the new kid on the block in systemic sclerosis? A systematic literature review and pilot study to evaluate reliability of LASCA to measure peripheral blood perfusion in scleroderma patients","volume":"17","author":"Cutolo","year":"2018","journal-title":"Autoimmun Rev"}],"container-title":["The Journal of Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.3899\/jrheum.2024-0103","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,9,1]],"date-time":"2024-09-01T12:30:18Z","timestamp":1725193818000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.jrheum.org\/lookup\/doi\/10.3899\/jrheum.2024-0103"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,6,15]]},"references-count":18,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2024,9,1]]},"published-print":{"date-parts":[[2024,9]]}},"alternative-id":["10.3899\/jrheum.2024-0103"],"URL":"https:\/\/doi.org\/10.3899\/jrheum.2024-0103","relation":{},"ISSN":["0315-162X","1499-2752"],"issn-type":[{"value":"0315-162X","type":"print"},{"value":"1499-2752","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,6,15]]}}}