{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T19:46:52Z","timestamp":1787687212474,"version":"build-2784847793"},"reference-count":28,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2018,9,17]],"date-time":"2018-09-17T00:00:00Z","timestamp":1537142400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis & Rheumatology"],"published-print":{"date-parts":[[2018,11]]},"abstract":"\n Objective<\/jats:title>\n \n There is a lack of agreement regarding treatment for many aspects of systemic sclerosis (\n SS<\/jats:styled-content>\n c). We undertook this study to generate\n SS<\/jats:styled-content>\n c treatment algorithms endorsed by a high percentage of\n SS<\/jats:styled-content>\n c experts.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (n = 170) were asked whether they agreed with\n SS<\/jats:styled-content>\n c algorithms from 2012. Two consensus rounds refined agreement; 62, 54, and 48 experts (36%, 32%, and 28%, respectively) completed the first, second, and third surveys, respectively.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n For treatment of scleroderma renal crisis, 81% of experts agreed (first\u2010, second\u2010, and third\u2010line treatments were angiotensin\u2010converting enzyme inhibitors, then adding calcium\u2010channel blockers [\n CCB<\/jats:styled-content>\n s], then adding angiotensin receptor blockers [\n ARB<\/jats:styled-content>\n s], respectively). For pulmonary arterial hypertension (\n PAH<\/jats:styled-content>\n ), 81% of experts agreed (for mild\n PAH<\/jats:styled-content>\n , treatments were phosphodiesterase 5 [\n PDE<\/jats:styled-content>\n 5] inhibitors, then endothelin receptor antagonists plus\n PDE<\/jats:styled-content>\n 5 inhibitors, then prostanoids, respectively; for severe\n PAH<\/jats:styled-content>\n , prostanoids were first\u2010line treatment). For mild Raynaud's phenomenon (\n RP<\/jats:styled-content>\n ), 79% of experts agreed (treatments were\n CCB<\/jats:styled-content>\n s, then adding\n PDE<\/jats:styled-content>\n 5 inhibitors, then\n ARB<\/jats:styled-content>\n s or switching to another\n CCB<\/jats:styled-content>\n , respectively; after the third line of treatment, mild\n RP<\/jats:styled-content>\n was deemed severe). For severe\n RP<\/jats:styled-content>\n , the first\u2010 through fourth\u2010line treatments were\n CCB<\/jats:styled-content>\n s, then adding\n PDE<\/jats:styled-content>\n 5 inhibitors or prostanoids, then adding\n PDE<\/jats:styled-content>\n 5 inhibitors (if not added as second\u2010line treatment) or prostanoids (if not added as second\u2010line treatment), then switching to another\n CCB<\/jats:styled-content>\n , respectively. For active treatment of digital ulcers, 66% of experts agreed (first\u2010 and second\u2010line treatments were\n CCB<\/jats:styled-content>\n s and\n PDE<\/jats:styled-content>\n 5 inhibitors, respectively). For interstitial lung disease, 69% of experts agreed (for induction therapy, treatments were mycophenolate mofetil [\n MMF<\/jats:styled-content>\n ], intravenous cyclophosphamide [\n IV CYC<\/jats:styled-content>\n ], and rituximab, respectively; for maintenance, first\u2010line treatment was\n MMF<\/jats:styled-content>\n ). For skin involvement, 71% of experts agreed (for a modified Rodnan skin thickness score [\n MRSS<\/jats:styled-content>\n ] of 24, first\u2010 and second\u2010line treatments were methotrexate [\n MTX<\/jats:styled-content>\n ] and\n MMF<\/jats:styled-content>\n , respectively; for an\n MRSS<\/jats:styled-content>\n of 32, first\u2010 through fourth\u2010line treatments were\n MMF<\/jats:styled-content>\n ,\n MTX<\/jats:styled-content>\n ,\n IV CYC<\/jats:styled-content>\n , and hematopoietic stem cell transplantation, respectively). For inflammatory arthritis, 79% of experts agreed (first\u2010 through fourth\u2010line treatments were\n MTX<\/jats:styled-content>\n , low\u2010dose glucocorticoids, hydroxychloroquine, and rituximab or tocilizumab, respectively). Algorithms for cardiac and gastrointestinal involvement had \u226575% agreement.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n \n Total agreement for\n SS<\/jats:styled-content>\n c algorithms was considerable. These algorithms may guide treatment.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/art.40560","type":"journal-article","created":{"date-parts":[[2018,5,21]],"date-time":"2018-05-21T06:37:09Z","timestamp":1526884629000},"page":"1820-1828","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":222,"title":["Treatment Algorithms for Systemic Sclerosis According to Experts"],"prefix":"10.1002","volume":"70","author":[{"given":"Andreu","family":"Fern\u00e1ndez\u2010Codina","sequence":"first","affiliation":[{"name":"University of Western Ontario, London, Ontario, Canada, and Hospital Universitari Vall d'Hebron Universitat Aut\u00f2noma de Barcelona Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kyle M.","family":"Walker","sequence":"additional","affiliation":[{"name":"The Ottawa Hospital University of Ottawa Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janet E.","family":"Pope","sequence":"additional","affiliation":[{"name":"University of Western Ontario London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Scleroderma Algorithm Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,9,17]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrdp.2015.2"},{"key":"e_1_2_6_3_1","first-page":"912","article-title":"Systemic sclerosis","volume":"56","author":"Denton CP","year":"2017","journal-title":"Lancet 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