{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T02:44:19Z","timestamp":1787021059696,"version":"build-2736575974"},"reference-count":95,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T00:00:00Z","timestamp":1720569600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T00:00:00Z","timestamp":1720569600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Adv Rheumatol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient\/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Six recommendations were elaborated regarding the pharmacological treatment of Raynaud\u2019s phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s42358-024-00392-w","type":"journal-article","created":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T06:01:46Z","timestamp":1720591306000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis"],"prefix":"10.1186","volume":"64","author":[{"given":"Cristiane","family":"Kayser","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra Maximiano","family":"de Oliveira Delgado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana Fontes","family":"Zimmermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex Magno Coelho","family":"Horimoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Paula Toledo","family":"Del Rio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolina","family":"de Souza M\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cintia Zumstein","family":"Camargo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristiano Michelini","family":"Lupo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela Aparecida","family":"de Moraes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo Jos\u00e9","family":"Do Ros\u00e1rio E Souza","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fl\u00e1via Patr\u00edcia Sena Teixeira","family":"Santos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juliana Yuri","family":"Sekiyama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lilian Scussel","family":"Lonzetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas Vict\u00f3ria","family":"de Oliveira Martins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mailze Campos","family":"Bezerra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Bredemeier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Carolina","family":"Oliveira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Cec\u00edlia","family":"da Fonseca Salgado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renata","family":"Miossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheila M\u00e1rcia","family":"de Ara\u00fajo Fontenele","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Hax","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea Tavares","family":"Dantas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Percival Degrava","family":"Sampaio-Barros","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,7,10]]},"reference":[{"issue":"10103","key":"392_CR1","doi-asserted-by":"publisher","first-page":"1685","DOI":"10.1016\/S0140-6736(17)30933-9","volume":"390","author":"CP Denton","year":"2017","unstructured":"Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017;390(10103):1685\u201399. https:\/\/doi.org\/10.1016\/S0140-6736(17)30933-9.","journal-title":"Lancet"},{"issue":"11","key":"392_CR2","doi-asserted-by":"publisher","first-page":"1897","DOI":"10.1136\/annrheumdis-2017-211448","volume":"76","author":"M Elhai","year":"2017","unstructured":"Elhai M, Meune C, Boubaya M, Avouac J, Hachulla E, Balbir-Gurman A, EUSTAR group, et al. Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis. 2017;76(11):1897\u2013905. https:\/\/doi.org\/10.1136\/annrheumdis-2017-211448.","journal-title":"Ann Rheum Dis"},{"issue":"8","key":"392_CR3","doi-asserted-by":"publisher","first-page":"1327","DOI":"10.1136\/annrheumdis-2016-209909","volume":"76","author":"O Kowal-Bielecka","year":"2017","unstructured":"Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, EUSTAR Coauthors, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327\u201339. https:\/\/doi.org\/10.1136\/annrheumdis-2016-209909.","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"392_CR4","doi-asserted-by":"publisher","first-page":"444","DOI":"10.1002\/1529-0131(200002)43:2<444::AID-ANR27>3.0.CO;2-G","volume":"43","author":"H Poormoghim","year":"2000","unstructured":"Poormoghim H, Lucas M, Fertig N, Medsger Jr TA. Systemic sclerosis sine scleroderma: demographic, clinical, and serologic features and survival in forty-eight patients. Arthritis Rheum. 2000;43(2):444\u201351.","journal-title":"Arthritis Rheum"},{"issue":"7","key":"392_CR5","first-page":"1573","volume":"28","author":"EC LeRoy","year":"2001","unstructured":"LeRoy EC, Medsger TA Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573\u201376.","journal-title":"J Rheumatol"},{"issue":"3","key":"392_CR6","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1136\/ard.2010.136929","volume":"70","author":"J Avouac","year":"2011","unstructured":"Avouac J, Fransen J, Walker UA, Riccieri V, Smith V, Muller C, EUSTAR Group, et al. Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. Ann Rheum Dis. 2011;70(3):476\u201381. https:\/\/doi.org\/10.1136\/ard.2010.136929.","journal-title":"Ann Rheum Dis"},{"key":"392_CR7","doi-asserted-by":"publisher","first-page":"102211","DOI":"10.1016\/j.coph.2022.102211","volume":"64","author":"H Bukiri","year":"2022","unstructured":"Bukiri H, Volkmann ER. Current advances in the treatment of systemic sclerosis. Curr Opin Pharmacol. 2022;64:102211. https:\/\/doi.org\/10.1016\/j.coph.2022.102211.","journal-title":"Curr Opin Pharmacol"},{"issue":"10373","key":"392_CR8","doi-asserted-by":"publisher","first-page":"304","DOI":"10.1016\/S0140-6736(22)01692-0","volume":"401","author":"ER Volkmann","year":"2023","unstructured":"Volkmann ER, Andr\u00e9asson K, Smith V. Systemic sclerosis. Lancet. 2023;401(10373):304\u201318. https:\/\/doi.org\/10.1016\/S0140-6736(22)01692-0.","journal-title":"Lancet"},{"issue":"1","key":"392_CR9","doi-asserted-by":"publisher","first-page":"2200879","DOI":"10.1183\/13993003.00879-2022","volume":"61","author":"M Humbert","year":"2023","unstructured":"Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, ESC\/ERS Scientific Document Group, et al. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023;61(1):2200879. https:\/\/doi.org\/10.1183\/13993003.00879-2022.","journal-title":"Eur Respir J"},{"issue":"4","key":"392_CR10","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1183\/13993003.01032-2015","volume":"46","author":"N Gali\u00e8","year":"2015","unstructured":"Gali\u00e8 N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the european society of cardiology (ESC) and the European respiratory society (ERS): endorsed by: association for European paediatric and congenital cardiology (AEPC), international society for heart and lung transplantation (ISHLT). Eur Respir J. 2015;46(4):903\u201375. https:\/\/doi.org\/10.1183\/13993003.01032-2015.","journal-title":"Eur Respir J"},{"key":"392_CR11","unstructured":"Brazil. Ministry of Health. Portaria no 10, from July 18, 2023. Protocolos Cl\u00ednicos e Diretrizes Terap\u00eauticas para Hipertens\u00e3o Pulmonar. Bras\u00edlia, 2023."},{"issue":"3","key":"392_CR12","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1590\/S0482-50042013000300004","volume":"53","author":"PD Sampaio-Barros","year":"2013","unstructured":"Sampaio-Barros PD, Zimmermann AF, de Souza M\u00fcller C, Borges CT, Freire EA, Maretti GB, Systemic sclerosis commission of the Brazilian society of rheumatology, et al. Recommendations for the management and treatment of systemic sclerosis. Rev Bras Reumatol. 2013;53(3):258\u201375.","journal-title":"Rev Bras Reumatol"},{"key":"392_CR13","unstructured":"Sch\u00fcnemann H, Bro\u017cek J, Guyatt G, Oxman A. GRADE handbook for grading quality of evidence and strength of recommendations (updated in October 2013). The GRADE Working Group; 2013. http:\/\/www.guidelinedevelopment.org\/handbook."},{"issue":"7001","key":"392_CR14","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1136\/bmj.311.7001.376","volume":"311","author":"J Jones","year":"1995","unstructured":"Jones J, Hunter D. Consensus methods for medical and health services research. BMJ. 1995;311(7001):376\u201380. https:\/\/doi.org\/10.1136\/bmj.311.7001.376.","journal-title":"BMJ"},{"key":"392_CR15","doi-asserted-by":"publisher","first-page":"n71","DOI":"10.1136\/bmj.n71","volume":"372","author":"MJ Page","year":"2021","unstructured":"Page MJ, McKenzie JE, Bossuyt PM, Boutron I, Hoffmann TC, Mulrow CD, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ. 2021;372:n71. https:\/\/doi.org\/10.1136\/bmj.n71.","journal-title":"BMJ"},{"issue":"1","key":"392_CR16","doi-asserted-by":"publisher","first-page":"210","DOI":"10.1186\/s13643-016-0384-4","volume":"5","author":"M Ouzzani","year":"2016","unstructured":"Ouzzani M, Hammady H, Fedorowicz Z, Elmagarmid A. Rayyan-a web and mobile app for systematic reviews. Syst Rev. 2016;5(1):210. https:\/\/doi.org\/10.1186\/s13643-016-0384-4.","journal-title":"Syst Rev"},{"key":"392_CR17","doi-asserted-by":"publisher","first-page":"e489","DOI":"10.1016\/S2665-9913(22)00131-X","volume":"3","author":"S Ebata","year":"2021","unstructured":"Ebata S, Yoshizaki A, Oba K, Kashiwabara K, Ueda K, Uemura Y, et al. Safety and efficacy of rituximab in systemic sclerosis (DESIRES): a double-blind, investigator-initiated, randomized, placebo-controlled trial. Lancet Rheumatol 2021;3:e489\u201397. https:\/\/doi.org\/10.1016\/S2665-9913(22)00131-X.","journal-title":"Lancet Rheumatol"},{"issue":"5","key":"392_CR18","doi-asserted-by":"publisher","first-page":"1936","DOI":"10.1093\/rheumatology\/keab584","volume":"61","author":"A Daumas","year":"2022","unstructured":"Daumas A, Magalon J, Jouve E, Casanova D, Philandrianos C, Abellan Lopez M, et al. Adipose tissue-derived stromal vascular fraction for treating hands of patients with systemic sclerosis: a multicentre randomized trial Autologous AD-SVF versus placebo in systemic sclerosis. Rheumatology (Oxford). 2022;61(5):1936\u201347. https:\/\/doi.org\/10.1093\/rheumatology\/keab584.","journal-title":"Rheumatology (Oxford)"},{"key":"392_CR19","unstructured":"Higgins JPT, Li T, Deeks JJ. Chapter 6: choosing effect measures and computing estimates of effect. In: Higgins JPT, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, Welch VA, editors. Cochrane handbook for systematic reviews of interventions version 6.3 (Updated February 2022). Cochrane, 2022. http:\/\/www.training.cochrane.org\/handbook."},{"issue":"12","key":"392_CR20","doi-asserted-by":"publisher","first-page":"2370","DOI":"10.1002\/art.40242","volume":"69","author":"CP Denton","year":"2017","unstructured":"Denton CP, Hachulla \u00c9, Riemekasten G, Schwarting A, Frenoux JM, Frey A, Raynaud Study Investigators, et al. Efficacy and safety of selexipag in adults with Raynaud\u2019s phenomenon secondary to systemic sclerosis: a randomized, placebo-controlled, phase II study. Arthritis Rheumatol. 2017;69(12):2370\u201379. https:\/\/doi.org\/10.1002\/art.40242.","journal-title":"Arthritis Rheumatol"},{"issue":"8","key":"392_CR21","doi-asserted-by":"publisher","first-page":"1661","DOI":"10.1002\/art.40123","volume":"69","author":"RJ Bello","year":"2017","unstructured":"Bello RJ, Cooney CM, Melamed E, Follmar K, Yenokyan G, Leatherman G, et al. The therapeutic efficacy of Botulinum toxin in treating scleroderma-associated Raynaud\u2019s phenomenon: a randomized, double-blind, placebo-controlled clinical trial. Arthritis Rheumatol. 2017;69(8):1661\u201369. https:\/\/doi.org\/10.1002\/art.40123.","journal-title":"Arthritis Rheumatol"},{"issue":"10","key":"392_CR22","doi-asserted-by":"publisher","first-page":"2264","DOI":"10.3899\/jrheum.090270","volume":"36","author":"E Schiopu","year":"2009","unstructured":"Schiopu E, Hsu VM, Impens AJ, Rothman JA, McCloskey DA, Wilson JE, et al. Randomized placebo-controlled crossover trial of tadalafil in Raynaud\u2019s phenomenon secondary to systemic sclerosis. J Rheumatol. 2009;36(10):2264\u201368. https:\/\/doi.org\/10.3899\/jrheum.090270.","journal-title":"J Rheumatol"},{"issue":"1","key":"392_CR23","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1093\/rheumatology\/key026","volume":"58","author":"JD Pauling","year":"2019","unstructured":"Pauling JD, Saketkoo LA, Matucci-Cerinic M, Ingegnoli F, Khanna D. The patient experience of Raynaud\u2019s phenomenon in systemic sclerosis. Rheumatology (Oxford). 2019;58(1):18\u201326. https:\/\/doi.org\/10.1093\/rheumatology\/key026.","journal-title":"Rheumatology (Oxford)"},{"issue":"1","key":"392_CR24","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1016\/j.semarthrit.2016.02.005","volume":"46","author":"C Frantz","year":"2016","unstructured":"Frantz C, Avouac J, Distler O, Amrouche F, Godard D, Kennedy AT, et al. Impaired quality of life in systemic sclerosis and patient perception of the disease: a large international survey. Semin Arthritis Rheum. 2016;46(1):115\u201323. https:\/\/doi.org\/10.1016\/j.semarthrit.2016.02.005.","journal-title":"Semin Arthritis Rheum"},{"key":"392_CR25","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1093\/eurheartj\/4.suppl_c.123","volume":"4 Suppl C","author":"A Kahan","year":"1983","unstructured":"Kahan A, Weber S, Amor B, Menkes CJ, Saporta L, Hodara M, et al. Calcium entry blocking agents in digital vasospasm (Raynaud\u2019s phenomenon). Eur Heart J. 1983;4 Suppl C:123\u201329. https:\/\/doi.org\/10.1093\/eurheartj\/4.suppl_c.123.","journal-title":"Eur Heart J"},{"issue":"8","key":"392_CR26","doi-asserted-by":"publisher","first-page":"702","DOI":"10.1093\/oxfordjournals.eurheartj.a061923","volume":"6","author":"A Kahan","year":"1985","unstructured":"Kahan A, Foult JM, Weber S, Amor B, Menkes CJ, Degeorges M. Nifedipine and alpha 1-adrenergic blockade in Raynaud\u2019s phenomenon. Eur Heart J. 1985;6(8):702\u201305. https:\/\/doi.org\/10.1093\/oxfordjournals.eurheartj.a061923.","journal-title":"Eur Heart J"},{"issue":"2","key":"392_CR27","doi-asserted-by":"publisher","first-page":"237","DOI":"10.1111\/j.1365-2133.1987.tb04122.x","volume":"117","author":"RH Meyrick Thomas","year":"1987","unstructured":"Meyrick Thomas RH, Rademaker M, Grimes SM, MacKay A, Kovacs IB, Cook ED, et al. Nifedipine in the treatment of Raynaud\u2019s phenomenon in patients with systemic sclerosis. Br J Dermatol. 1987;117(2):237\u201341. https:\/\/doi.org\/10.1111\/j.1365-2133.1987.tb04122.x.","journal-title":"Br J Dermatol"},{"issue":"15","key":"392_CR28","doi-asserted-by":"publisher","first-page":"880","DOI":"10.1056\/NEJM198304143081507","volume":"308","author":"RJ Rodeheffer","year":"1983","unstructured":"Rodeheffer RJ, Rommer JA, Wigley F, Smith CR. Controlled double-blind trial of nifedipine in the treatment of Raynaud\u2019s phenomenon. N Engl J Med. 1983;308(15):880\u201383. https:\/\/doi.org\/10.1056\/NEJM198304143081507.","journal-title":"N Engl J Med"},{"key":"392_CR29","first-page":"2086","volume":"62 Suppl 10","author":"V Agarwal","year":"2010","unstructured":"Agarwal V, Ghosh P, Sharma A, Bhakuni DS, Kumar S, Singh UN, et al. Efficacy of Tadalafil in Raynaud\u2019s phenomenon secondary to systemic sclerosis: a double blind randomized placebo controlled parallel group multicentric study. Arthritis Rheum. 2010;62 Suppl 10:2086. [ABSTRACT]","journal-title":"Arthritis Rheum"},{"issue":"4","key":"392_CR30","first-page":"151","volume":"35 Suppl 106","author":"FV Andrigueti","year":"2017","unstructured":"Andrigueti FV, Ebbing PCC, Arismendi MI, Kayser C. Evaluation of the effect of sildenafil on the microvascular blood flow in patients with systemic sclerosis: a randomised, double-blind, placebo-controlled study. Clin Exp Rheumatol. 2017;35 Suppl 106(4):151\u201358.","journal-title":"Clin Exp Rheumatol"},{"issue":"15","key":"392_CR31","doi-asserted-by":"publisher","first-page":"1182","DOI":"10.1001\/archinternmed.2012.2271","volume":"172","author":"E Caglayan","year":"2012","unstructured":"Caglayan E, Axmann S, Hellmich M, Moinzadeh P, Rosenkranz S. Vardenafil for the treatment of Raynaud phenomenon: a randomized, double-blind, placebo-controlled crossover study. Arch Intern Med. 2012;172(15):1182\u201384. https:\/\/doi.org\/10.1001\/archinternmed.2012.2271.","journal-title":"Arch Intern Med"},{"issue":"19","key":"392_CR32","doi-asserted-by":"publisher","first-page":"2980","DOI":"10.1161\/CIRCULATIONAHA.104.523324","volume":"112","author":"R Fries","year":"2005","unstructured":"Fries R, Shariat K, von Wilmowsky H, B\u00f6hm M. Sildenafil in the treatment of Raynaud\u2019s phenomenon resistant to vasodilatory therapy. Circulation. 2005;112(19):2980\u201385. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.104.523324.","journal-title":"Circulation"},{"issue":"3","key":"392_CR33","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1002\/art.30195","volume":"63","author":"AL Herrick","year":"2011","unstructured":"Herrick AL, van den Hoogen F, Gabrielli A, Tamimi N, Reid C, O\u2019Connell D, et al. Modified-release sildenafil reduces Raynaud\u2019s phenomenon attack frequency in limited cutaneous systemic sclerosis. Arthritis Rheum. 2011;63(3):775\u201382. https:\/\/doi.org\/10.1002\/art.30195.","journal-title":"Arthritis Rheum"},{"issue":"12","key":"392_CR34","doi-asserted-by":"publisher","first-page":"2420","DOI":"10.1093\/rheumatology\/keq291","volume":"49","author":"PD Shenoy","year":"2010","unstructured":"Shenoy PD, Kumar S, Jha LK, Choudhary SK, Singh U, Misra R, et al. Efficacy of tadalafil in secondary Raynaud\u2019s phenomenon resistant to vasodilator therapy: a double-blind randomized cross-over trial. Rheumatology (Oxford). 2010;49(12):2420\u201328. https:\/\/doi.org\/10.1093\/rheumatology\/keq291.","journal-title":"Rheumatology (Oxford)"},{"issue":"6","key":"392_CR35","doi-asserted-by":"publisher","first-page":"1009","DOI":"10.1136\/annrheumdis-2014-207001","volume":"75","author":"E Hachulla","year":"2016","unstructured":"Hachulla E, Hatron PY, Carpentier P, Agard C, Chatelus E, Jego P, SEDUCE study group, et al. Efficacy of sildenafil on ischaemic digital ulcer healing in systemic sclerosis: the placebo-controlled SEDUCE study. Ann Rheum Dis. 2016;75(6):1009\u201315. https:\/\/doi.org\/10.1136\/annrheumdis-2014-207001.","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"392_CR36","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1136\/ard.47.1.43","volume":"47","author":"NJ McHugh","year":"1988","unstructured":"McHugh NJ, Csuka M, Watson H, Belcher G, Amadi A, Ring EF, et al. Infusion of iloprost, a prostacyclin analogue, for treatment of Raynaud\u2019s phenomenon in systemic sclerosis. Ann Rheum Dis. 1988;47(1):43\u201347. https:\/\/doi.org\/10.1136\/ard.47.1.43.","journal-title":"Ann Rheum Dis"},{"issue":"9","key":"392_CR37","first-page":"1407","volume":"19","author":"FM Wigley","year":"1992","unstructured":"Wigley FM, Seibold JR, Wise RA, McCloskey DA, Dole WP. Intravenous iloprost treatment of Raynaud\u2019s phenomenon and ischemic ulcers secondary to systemic sclerosis. J Rheumatol. 1992;19(9):1407\u201314.","journal-title":"J Rheumatol"},{"issue":"3","key":"392_CR38","doi-asserted-by":"publisher","first-page":"199","DOI":"10.7326\/0003-4819-120-3-199402010-00004","volume":"120","author":"FM Wigley","year":"1994","unstructured":"Wigley FM, Wise RA, Seibold JR, McCloskey DA, Kujala G, Medsger TA Jr, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med. 1994;120(3):199\u2013206. https:\/\/doi.org\/10.7326\/0003-4819-120-3-199402010-00004.","journal-title":"Ann Intern Med"},{"issue":"9","key":"392_CR39","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1093\/rheumatology\/37.9.952","volume":"37","author":"CM Black","year":"1998","unstructured":"Black CM, Halkier-S\u00f8rensen L, Belch JJ, Ullman S, Madhok R, Smit AJ, et al. Oral iloprost in Raynaud\u2019s phenomenon secondary to systemic sclerosis: a multicentre, placebo-controlled, dose-comparison study. Br J Rheumatol. 1998;37(9):952\u201360. https:\/\/doi.org\/10.1093\/rheumatology\/37.9.952.","journal-title":"Br J Rheumatol"},{"issue":"4","key":"392_CR40","doi-asserted-by":"publisher","first-page":"670","DOI":"10.1002\/1529-0131(199804)41:4<670::AID-ART14>;3.0.CO;2-I","volume":"41","author":"FM Wigley","year":"1998","unstructured":"Wigley FM, Korn JH, Csuka ME, Medsger Jr TA, Rothfield NF, Ellman M, et al. Oral iloprost treatment in patients with Raynaud\u2019s phenomenon secondary to systemic sclerosis: a multicenter, placebo-controlled, double-blind study. Arthritis Rheum. 1998;41(4):670\u201377. https:\/\/doi.org\/10.1002\/1529-0131(199804)41:4<670::AID-ART14>;3.0.CO;2-I.","journal-title":"Arthritis Rheum"},{"issue":"3","key":"392_CR41","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1136\/ard.54.3.197","volume":"54","author":"JJ Belch","year":"1995","unstructured":"Belch JJ, Capell HA, Cooke ED, Kirby JD, Lau CS, Madhok R, et al. Oral iloprost as a treatment for Raynaud\u2019s syndrome: a double-blind multicentre placebo-controlled study. Ann Rheum Dis. 1995;54(3):197\u2013200. https:\/\/doi.org\/10.1136\/ard.54.3.197.","journal-title":"Ann Rheum Dis"},{"issue":"4","key":"392_CR42","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1080\/03009740310004711","volume":"33","author":"B Marasini","year":"2004","unstructured":"Marasini B, Massarotti M, Bottasso B, Coppola R, Del Papa N, Maglione W, Comina DP, Maioli C. Comparison between iloprost and alprostadil in the treatment of Raynaud\u2019s phenomenon. Scand J Rheumatol. 2004;33(4):253\u201356. https:\/\/doi.org\/10.1080\/03009740310004711.","journal-title":"Scand J Rheumatol"},{"key":"392_CR43","first-page":"1505","volume":"71 Suppl 10","author":"R Domsic","year":"2019","unstructured":"Domsic R, Laffoon M, Goundappa B, Medsger T, Lafyatis R, Wisniewski S. The effect of atorvastatin on microvascular endothelial function and Raynaud phenomenon in early diffuse scleroderma: results of the \u201ctamer\u201d study. Arthritis Rheum. 2019;71 Suppl 10:1505\u201306. [ABSTRACT].","journal-title":"Arthritis Rheum"},{"issue":"3","key":"392_CR44","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1093\/rheumatology\/kep413","volume":"49","author":"VA Nguyen","year":"2010","unstructured":"Nguyen VA, Eisendle K, Gruber I, Hugl B, Reider D, Reider N. Effect of the dual endothelin receptor antagonist bosentan on Raynaud\u2019s phenomenon secondary to systemic sclerosis: a double-blind prospective, randomized, placebo-controlled pilot study. Rheumatology (Oxford). 2010;49(3):583\u201387. https:\/\/doi.org\/10.1093\/rheumatology\/kep413.","journal-title":"Rheumatology (Oxford)"},{"issue":"4","key":"392_CR45","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1038\/s41584-020-0386-4","volume":"16","author":"M Hughes","year":"2020","unstructured":"Hughes M, Allanore Y, Chung L, Pauling JD, Denton CP, Matucci-Cerinic M. Raynaud phenomenon and digital ulcers in systemic sclerosis. Nat Rev Rheumatol. 2020;16(4):208\u201321. https:\/\/doi.org\/10.1038\/s41584-020-0386-4.","journal-title":"Nat Rev Rheumatol"},{"issue":"4","key":"392_CR46","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1136\/annrheumdis-2014-205897","volume":"75","author":"C Mihai","year":"2016","unstructured":"Mihai C, Landew\u00e9 R, van der Heijde D, Walker UA, Constantin PI, Gherghe AM, EUSTAR co-authors, et al. Digital ulcers predict a worse disease course in patients with systemic sclerosis. Ann Rheum Dis. 2016;75(4):681\u201386. https:\/\/doi.org\/10.1136\/annrheumdis-2014-205897.","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"392_CR47","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/s13075-018-1792-8","volume":"21","author":"N Del Papa","year":"2019","unstructured":"Del Papa N, Di Luca G, Andracco R, Zaccara E, Maglione W, Pignataro F, et al. Regional grafting of autologous adipose tissue is effective in inducing prompt healing of indolent digital ulcers in patients with systemic sclerosis: results of a monocentric randomized controlled study. Arthritis Res Ther. 2019;21(1):7. https:\/\/doi.org\/10.1186\/s13075-018-1792-8.","journal-title":"Arthritis Res Ther"},{"issue":"18","key":"392_CR48","doi-asserted-by":"publisher","first-page":"1975","DOI":"10.1001\/jama.2016.5258","volume":"315","author":"D Khanna","year":"2016","unstructured":"Khanna D, Denton CP, Merkel PA, Krieg T, Le Brun FO, Marr A, DUAL-1 Investigators, DUAL-2 Investigators, et al. Effect of macitentan on the development of new ischemic digital ulcers in patients with systemic sclerosis: DUAL-1 and DUAL-2 randomized clinical trials. JAMA. 2016;315(18):1975\u201388. https:\/\/doi.org\/10.1001\/jama.2016.5258.","journal-title":"JAMA"},{"issue":"9","key":"392_CR49","first-page":"1801","volume":"35","author":"A Abou-Raya","year":"2008","unstructured":"Abou-Raya A, Abou-Raya S, Helmii M. Statins: potentially useful in therapy of systemic sclerosis-related Raynaud\u2019s phenomenon and digital ulcers. J Rheumatol. 2008;35(9):1801\u201308.","journal-title":"J Rheumatol"},{"issue":"12","key":"392_CR50","doi-asserted-by":"publisher","first-page":"3985","DOI":"10.1002\/art.20676","volume":"50","author":"JH Korn","year":"2004","unstructured":"Korn JH, Mayes M, Matucci-Cerinic M, Rainisio M, Pope J, Hachulla E, et al. Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist. Arthritis Rheum. 2004;50(12):3985\u201393. https:\/\/doi.org\/10.1002\/art.20676.","journal-title":"Arthritis Rheum"},{"issue":"1","key":"392_CR51","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1136\/ard.2010.130658","volume":"70","author":"M Matucci-Cerinic","year":"2011","unstructured":"Matucci-Cerinic M, Denton CP, Furst DE, Mayes MD, Hsu VM, Carpentier P, et al. Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomized, double-blind, placebo-controlled trial. Ann Rheum Dis. 2011;70(1):32\u201338. https:\/\/doi.org\/10.1136\/ard.2010.130658.","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"392_CR52","doi-asserted-by":"publisher","first-page":"e002890","DOI":"10.1136\/rmdopen-2022-002890","volume":"9","author":"R De Angelis","year":"2023","unstructured":"De Angelis R, Ferri C, Giuggioli D, Bajocchi G, Dagna L, Bellando-Randone S, SPRING-SIR (Systemic Sclerosis PRogression INvestiGation group of the Italian Society of Rheumatology), et al. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry \u2018SPRING\u2019 of the Italian Society for Rheumatology. RMD Open. 2023;9(1):e002890. https:\/\/doi.org\/10.1136\/rmdopen-2022-002890.","journal-title":"RMD Open"},{"issue":"1","key":"392_CR53","doi-asserted-by":"publisher","first-page":"104","DOI":"10.1136\/ard.2009.127621","volume":"70","author":"RT Domsic","year":"2011","unstructured":"Domsic RT, Rodriguez-Reyna T, Lucas M, Fertig N, Medsger TA Jr. Skin thickness progression rate: a predictor of mortality and early internal organ involvement in diffuse scleroderma. Ann Rheum Dis. 2011;70(1):104\u201309. https:\/\/doi.org\/10.1136\/ard.2009.127621.","journal-title":"Ann Rheum Dis"},{"issue":"5","key":"392_CR54","doi-asserted-by":"publisher","first-page":"276","DOI":"10.1038\/s41584-022-00765-9","volume":"18","author":"AL Herrick","year":"2022","unstructured":"Herrick AL, Assassi S, Denton CP. Skin involvement in early diffuse cutaneous systemic sclerosis: an unmet clinical need. Nat Rev Rheumatol. 2022;18(5):276\u201385. https:\/\/doi.org\/10.1038\/s41584-022-00765-9.","journal-title":"Nat Rev Rheumatol"},{"issue":"6","key":"392_CR55","doi-asserted-by":"publisher","first-page":"1351","DOI":"10.1002\/1529-0131(200106)44:6<1351::AID-ART227>3.0.CO;2-I","volume":"44","author":"JE Pope","year":"2001","unstructured":"Pope JE, Bellamy N, Seibold JR, Baron M, Ellman M, Carette S, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum. 2001;44(6):1351\u201358. https:\/\/doi.org\/10.1002\/1529-0131(200106)44:6<1351::AID-ART227>3.0.CO;2-I.","journal-title":"Arthritis Rheum"},{"issue":"4","key":"392_CR56","doi-asserted-by":"publisher","first-page":"364","DOI":"10.1093\/rheumatology\/35.4.364","volume":"35","author":"FH van den Hoogen","year":"1996","unstructured":"van den Hoogen FH, Boerbooms AM, Swaak AJ, Rasker JJ, van Lier HJ, van de Putte LB. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24-week randomized double-blind trial, followed by a 24-week observational trial. Br J Rheumatol. 1996;35(4):364\u201372. https:\/\/doi.org\/10.1093\/rheumatology\/35.4.364.","journal-title":"Br J Rheumatol"},{"issue":"25","key":"392_CR57","doi-asserted-by":"publisher","first-page":"2655","DOI":"10.1056\/NEJMoa055120","volume":"354","author":"DP Tashkin","year":"2006","unstructured":"Tashkin DP, Elashoff R, Clements PJ, Goldin J, Roth MD, Furst DE, Scleroderma Lung Study Research Group, et al. Cyclophosphamide versus placebo in scleroderma lung disease. N Engl J Med. 2006;354(25):2655\u201366. https:\/\/doi.org\/10.1056\/NEJMoa055120.","journal-title":"N Engl J Med"},{"issue":"2","key":"392_CR58","doi-asserted-by":"publisher","first-page":"205","DOI":"10.1007\/s10067-005-1157-y","volume":"25","author":"O Nadashkevich","year":"2006","unstructured":"Nadashkevich O, Davis P, Fritzler M, Kovalenko W. A randomized unblinded trial of cyclophosphamide versus azathioprine in the treatment of systemic sclerosis. Clin Rheumatol. 2006;25(2):205\u201312. https:\/\/doi.org\/10.1007\/s10067-005-1157-y.","journal-title":"Clin Rheumatol"},{"issue":"2","key":"392_CR59","doi-asserted-by":"publisher","first-page":"207","DOI":"10.1007\/s00296-019-04481-8","volume":"40","author":"GSRSNK Naidu","year":"2020","unstructured":"Naidu GSRSNK, Sharma SK, Adarsh MB, Dhir V, Sinha A, Dhooria S, Jain S. Effect of mycophenolate mofetil (MMF) on systemic sclerosis-related interstitial lung disease with mildly impaired lung function: a double-blind, placebo-controlled, randomized trial. Rheumatol Int. 2020;40(2):207\u201316. https:\/\/doi.org\/10.1007\/s00296-019-04481-8.","journal-title":"Rheumatol Int"},{"issue":"9","key":"392_CR60","doi-asserted-by":"publisher","first-page":"708","DOI":"10.1016\/S2213-2600(16)30152-7","volume":"4","author":"DP Tashkin","year":"2016","unstructured":"Tashkin DP, Roth MD, Clements PJ, Furst DE, Khanna D, Kleerup EC, Sclerodema Lung Study II Investigators, et al. Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomized controlled, double-blind, parallel group trial. Lancet Respir Med. 2016;4(9):708\u201319. https:\/\/doi.org\/10.1016\/S2213-2600(16)30152-7.","journal-title":"Lancet Respir Med"},{"issue":"9790","key":"392_CR61","doi-asserted-by":"publisher","first-page":"498","DOI":"10.1016\/S0140-6736(11)60982-3","volume":"378","author":"RK Burt","year":"2011","unstructured":"Burt RK, Shah SJ, Dill K, Grant T, Gheorghiade M, Schroeder J, et al. Autologous non-myeloablative haemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomized phase 2 trial. Lancet. 2011;378(9790):498\u2013506. https:\/\/doi.org\/10.1016\/S0140-6736(11)60982-3.","journal-title":"Lancet"},{"key":"392_CR62","doi-asserted-by":"publisher","unstructured":"Sullivan KM, Goldmuntz EA, Keyes-Elstein L, McSweeney PA, Pinckney A, Welch B, SCOT Study Investigators, et al. Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med. 378(1):35\u201347. https:\/\/doi.org\/10.1056\/nejmoa1703327.","DOI":"10.1056\/nejmoa1703327"},{"issue":"24","key":"392_CR63","doi-asserted-by":"publisher","first-page":"2490","DOI":"10.1001\/jama.2014.6368","volume":"311","author":"JM van Laar","year":"2014","unstructured":"van Laar JM, Farge D, Sont JK, Naraghi K, Marjanovic Z, Larghero J, EBMT\/EULAR Scleroderma Study Group, et al. Autologous hematopoietic stem cell transplantation vs intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis: a randomized clinical trial. JAMA. 2014;311(24):2490\u201398. https:\/\/doi.org\/10.1001\/jama.2014.6368.","journal-title":"JAMA"},{"issue":"2","key":"392_CR64","doi-asserted-by":"publisher","first-page":"e000384","DOI":"10.1136\/rmdopen-2016-000384","volume":"3","author":"M Boonstra","year":"2017","unstructured":"Boonstra M, Meijs J, Dorj\u00e9e AL, Marsan NA, Schouffoer A, Ninaber MK, et al. Rituximab in early systemic sclerosis. RMD Open. 2017;3(2):e000384. https:\/\/doi.org\/10.1136\/rmdopen-2016-000384.","journal-title":"RMD Open"},{"issue":"2","key":"392_CR65","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1093\/rheumatology\/kep093","volume":"49","author":"D Daoussis","year":"2010","unstructured":"Daoussis D, Liossis SN, Tsamandas AC, Kalogeropoulou C, Kazantzi A, Sirinian C, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49(2):271\u201380. https:\/\/doi.org\/10.1093\/rheumatology\/kep093.","journal-title":"Rheumatology (Oxford)"},{"issue":"12","key":"392_CR66","doi-asserted-by":"publisher","first-page":"2106","DOI":"10.1093\/rheumatology\/key213","volume":"57","author":"G Sircar","year":"2018","unstructured":"Sircar G, Goswami RP, Sircar D, Ghosh A, Ghosh P. Intravenous cyclophosphamide vs rituximab for the treatment of early diffuse scleroderma lung disease: open label, randomized, controlled trial. Rheumatology (Oxford). 2018;57(12):2106\u201313. https:\/\/doi.org\/10.1093\/rheumatology\/key213.","journal-title":"Rheumatology (Oxford)"},{"issue":"10038","key":"392_CR67","doi-asserted-by":"publisher","first-page":"2630","DOI":"10.1016\/S0140-6736(16)00232-4","volume":"387","author":"D Khanna","year":"2016","unstructured":"Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, et al. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomized, controlled trial. Lancet. 2016;387(10038):2630\u201340. https:\/\/doi.org\/10.1016\/S0140-6736(16)00232-4.","journal-title":"Lancet"},{"issue":"10","key":"392_CR68","doi-asserted-by":"publisher","first-page":"963","DOI":"10.1016\/S2213-2600(20)30318-0","volume":"8","author":"D Khanna","year":"2020","unstructured":"Khanna D, Lin CJF, Furst DE, Goldin J, Kim G, Kuwana M, focuSSced investigators, et al. Tocilizumab in systemic sclerosis: a randomized, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med. 2020;8(10):963\u201374. https:\/\/doi.org\/10.1016\/S2213-2600(20)30318-0.","journal-title":"Lancet Respir Med"},{"issue":"1","key":"392_CR69","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1186\/s13075-015-0669-3","volume":"17","author":"EF Chakravarty","year":"2015","unstructured":"Chakravarty EF, Martyanov V, Fiorentino D, Wood TA, Haddon DJ, Jarrell JA, et al. Gene expression changes reflect clinical response in a placebo-controlled randomized trial of abatacept in patients with diffuse cutaneous systemic sclerosis. Arthritis Res Ther. 2015;17(1):159. https:\/\/doi.org\/10.1186\/s13075-015-0669-3.","journal-title":"Arthritis Res Ther"},{"issue":"1","key":"392_CR70","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1002\/art.41055","volume":"72","author":"D Khanna","year":"2020","unstructured":"Khanna D, Spino C, Johnson S, Chung L, Whitfield ML, Denton CP, et al. Abatacept in early diffuse cutaneous systemic sclerosis: results of a phase II investigator-initiated, multicenter, double-blind, randomized, placebo-controlled trial. Arthritis Rheumatol. 2020;72(1):125\u201336. https:\/\/doi.org\/10.1002\/art.41055.","journal-title":"Arthritis Rheumatol"},{"issue":"26","key":"392_CR71","doi-asserted-by":"publisher","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","volume":"380","author":"O Distler","year":"2019","unstructured":"Distler O, Highland KB, Gahlemann M, Azuma A, Fischer A, Mayes MD, SENSCIS Trial Investigators, et al. Nintedanib for systemic sclerosis-associated interstitial lung disease. N Engl J Med. 2019;380(26):2518\u201328. https:\/\/doi.org\/10.1056\/NEJMoa1903076.","journal-title":"N Engl J Med"},{"issue":"5","key":"392_CR72","doi-asserted-by":"publisher","first-page":"703","DOI":"10.1007\/s00296-020-04565-w","volume":"40","author":"N Acharya","year":"2020","unstructured":"Acharya N, Sharma SK, Mishra D, Dhooria S, Dhir V, Jain S. Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease-a randomized controlled trial. Rheumatol Int. 2020;40(5):703\u201310. https:\/\/doi.org\/10.1007\/s00296-020-04565-w.","journal-title":"Rheumatol Int"},{"issue":"10","key":"392_CR73","doi-asserted-by":"publisher","first-page":"1971","DOI":"10.3899\/jrheum.111582","volume":"39","author":"PD Sampaio-Barros","year":"2012","unstructured":"Sampaio-Barros PD, Bortoluzzo AB, Marangoni RG, Rocha LF, Del Rio AP, Samara AM, et al. Survival, causes of death, and prognostic factors in systemic sclerosis: analysis of 947 Brazilian patients. J Rheumatol. 2012;39(10):1971\u201378. https:\/\/doi.org\/10.3899\/jrheum.111582.","journal-title":"J Rheumatol"},{"issue":"2","key":"392_CR74","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1136\/annrheumdis-2020-217455","volume":"80","author":"AM Hoffmann-Vold","year":"2021","unstructured":"Hoffmann-Vold AM, Allanore Y, Alves M, Brunborg C, Air\u00f3 P, Ananieva LP, EUSTAR collaborators, et al. Progressive interstitial lung disease in patients with systemic sclerosis-associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2021;80(2):219\u201327. https:\/\/doi.org\/10.1136\/annrheumdis-2020-217455.","journal-title":"Ann Rheum Dis"},{"issue":"51","key":"392_CR75","doi-asserted-by":"publisher","first-page":"e8980","DOI":"10.1097\/MD.0000000000008980","volume":"96","author":"ND Morgan","year":"2017","unstructured":"Morgan ND, Shah AA, Mayes MD, Domsic RT, Medsger Jr TA, Steen VD, et al. Clinical and serological features of systemic sclerosis in a multicenter African American cohort: analysis of the genome research in African American scleroderma patient\u2019s clinical database. Medicine (Baltimore). 2017;96(51):e8980. https:\/\/doi.org\/10.1097\/MD.0000000000008980.","journal-title":"Medicine (Baltimore)"},{"issue":"1","key":"392_CR76","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1007\/s10067-019-04575-5","volume":"39","author":"C Mendes","year":"2020","unstructured":"Mendes C, Viana VST, Pasoto SG, Leon EP, Bonfa E, Sampaio-Barros PD. Clinical and laboratory features of African-Brazilian patients with systemic sclerosis. Clin Rheumatol. 2020;39(1):9\u201317. https:\/\/doi.org\/10.1007\/s10067-019-04575-5.","journal-title":"Clin Rheumatol"},{"issue":"11","key":"392_CR77","doi-asserted-by":"publisher","first-page":"1747","DOI":"10.1136\/annrheumdis-2013-204424","volume":"72","author":"F van den Hoogen","year":"2013","unstructured":"van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology\/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747\u201355. https:\/\/doi.org\/10.1136\/annrheumdis-2013-204424.","journal-title":"Ann Rheum Dis"},{"issue":"4","key":"392_CR78","doi-asserted-by":"publisher","first-page":"212","DOI":"10.1038\/s41584-023-00909-5","volume":"19","author":"JE Pope","year":"2023","unstructured":"Pope JE, Denton CP, Johnson SR, Fernandez-Codina A, Hudson M, Nevskaya T. State-of-the-art evidence in the treatment of systemic sclerosis. Nat Rev Rheumatol. 2023;19(4):212\u201326.","journal-title":"Nat Rev Rheumatol"},{"issue":"1","key":"392_CR79","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1186\/s12931-020-01459-0","volume":"21","author":"AM Hoffmann-Vold","year":"2020","unstructured":"Hoffmann-Vold AM, Allanore Y, Bendstrup E, Bruni C, Distler O, Maher TM, et al. The need for a holistic approach for SSc-ILD\u2013 achievements and ambiguity in a devastating disease. Respir Res. 2020;21(1):197.","journal-title":"Respir Res"},{"issue":"3","key":"392_CR80","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1038\/s41584-022-00900-6","volume":"19","author":"ZH McMahan","year":"2023","unstructured":"McMahan ZH, Kulkarni S, Chen J, Chen JZ, Xavier RJ, Pasricha PJ, Khanna D. Systemic sclerosis gastrointestinal dysmotility: risk factors, pathophysiology, diagnosis and management. Nat Rev Rheumatol. 2023;19(3):166\u201381. https:\/\/doi.org\/10.1038\/s41584-022-00900-6.","journal-title":"Nat Rev Rheumatol"},{"issue":"2","key":"392_CR81","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1016\/j.rdc.2023.01.006","volume":"49","author":"A Quinlivan","year":"2023","unstructured":"Quinlivan A, McMahan ZH, Lee EB, Nikpour M. Gastrointestinal tract considerations part I: how should a rheumatologist best manage common upper gastrointestinal tract complaints in systemic sclerosis? Rheum Dis Clin North Am. 2023;49(2):295\u2013318. https:\/\/doi.org\/10.1016\/j.rdc.2023.01.006.","journal-title":"Rheum Dis Clin North Am"},{"issue":"6","key":"392_CR82","doi-asserted-by":"publisher","first-page":"328","DOI":"10.1097\/BOR.0000000000000899","volume":"34","author":"ER Volkmann","year":"2022","unstructured":"Volkmann ER, McMahan Z. Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment. Curr Opin Rheumatol. 2022;34(6):328\u201336. https:\/\/doi.org\/10.1097\/BOR.0000000000000899.","journal-title":"Curr Opin Rheumatol"},{"issue":"1","key":"392_CR83","doi-asserted-by":"publisher","first-page":"145","DOI":"10.1186\/s13075-017-1340-y","volume":"19","author":"B Vigone","year":"2017","unstructured":"Vigone B, Caronni M, Severino A, Bellocchi C, Baldassarri AR, Fraquelli M, et al. Preliminary safety and efficacy profile of prucalopride in the treatment of systemic sclerosis (SSc)-related intestinal involvement: results from the open label cross-over PROGASS study. Arthritis Res Ther. 2017;19(1):145. https:\/\/doi.org\/10.1186\/s13075-017-1340-y.","journal-title":"Arthritis Res Ther"},{"issue":"3","key":"392_CR84","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1016\/j.semarthrit.2019.05.006","volume":"49","author":"AHL Low","year":"2019","unstructured":"Low AHL, Teng GG, Pettersson S, de Sessions PF, EXP H, Fan Q, et al. A double-blind randomized placebo-controlled trial of probiotics in systemic sclerosis associated gastrointestinal disease. Semin Arthritis Rheum. 2019;49(3):411\u201319. https:\/\/doi.org\/10.1016\/j.semarthrit.2019.05.006.","journal-title":"Semin Arthritis Rheum"},{"issue":"11","key":"392_CR85","doi-asserted-by":"publisher","first-page":"1985","DOI":"10.1093\/rheumatology\/kez160","volume":"58","author":"TF Marighela","year":"2019","unstructured":"Marighela TF, Arismendi MI, Marvulle V, Brunialti MKC, Salom\u00e3o R, Kayser C. Effect of probiotics on gastrointestinal symptoms and immune parameters in systemic sclerosis: a randomized placebo-controlled trial. Rheumatology (Oxford). 2019;58(11):1985\u201390. https:\/\/doi.org\/10.1093\/rheumatology\/kez160.","journal-title":"Rheumatology (Oxford)"},{"key":"392_CR86","doi-asserted-by":"publisher","first-page":"iii32","DOI":"10.1093\/rheumatology\/ken483","volume":"48 Suppl 3","author":"CP Denton","year":"2009","unstructured":"Denton CP, Lapadula G, Mouthon L, M\u00fcller-Ladner U. Renal complications and scleroderma renal crisis. Rheumatology (Oxford). 2009;48 Suppl 3:iii32\u20135. https:\/\/doi.org\/10.1093\/rheumatology\/ken483.","journal-title":"Rheumatology (Oxford)"},{"issue":"3","key":"392_CR87","doi-asserted-by":"publisher","first-page":"101707","DOI":"10.1016\/j.berh.2021.101707","volume":"35","author":"LA Saketkoo","year":"2021","unstructured":"Saketkoo LA, Frech T, Varj\u00fa C, Domsic R, Farrell J, Gordon JK, et al. A comprehensive framework for navigating patient care in systemic sclerosis: a global response to the need for improving the practice of diagnostic and preventive strategies in SSc. Best Pract Res Clin Rheumatol. 2021;35(3):101707. https:\/\/doi.org\/10.1016\/j.berh.2021.101707.","journal-title":"Best Pract Res Clin Rheumatol"},{"issue":"5","key":"392_CR88","doi-asserted-by":"publisher","first-page":"352","DOI":"10.7326\/0003-4819-113-5-352","volume":"113","author":"VD Steen","year":"1990","unstructured":"Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med. 1990;113(5):352\u201357. https:\/\/doi.org\/10.7326\/0003-4819-113-5-352.","journal-title":"Ann Intern Med"},{"issue":"7","key":"392_CR89","doi-asserted-by":"publisher","first-page":"940","DOI":"10.1136\/ard.2006.066068","volume":"66","author":"VD Steen","year":"2007","unstructured":"Steen VD, Medsger TA. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis. 2007;66(7):940\u201344. https:\/\/doi.org\/10.1136\/ard.2006.066068.","journal-title":"Ann Rheum Dis"},{"issue":"2","key":"392_CR90","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1016\/j.rdc.2023.01.008","volume":"49","author":"C Kayser","year":"2023","unstructured":"Kayser C, Vict\u00f3ria de Oliveira Martins L. Treatment of inflammatory arthritis in systemic sclerosis. Rheum Dis Clin North Am. 2023;49(2):337\u201343. https:\/\/doi.org\/10.1016\/j.rdc.2023.01.008.","journal-title":"Rheum Dis Clin North Am"},{"issue":"3","key":"392_CR91","first-page":"CD000189","volume":"2005","author":"PC Gotzsche","year":"2004","unstructured":"Gotzsche PC, Johansen HK. Short-term low-dose corticosteroids vs placebo and nonsteroidal antiinflammatory drugs in rheumatoid arthritis. Cochrane Database Syst Rev. 2004;2005(3):CD000189.","journal-title":"Cochrane Database Syst Rev"},{"issue":"10 Pt 2","key":"392_CR92","doi-asserted-by":"publisher","first-page":"e315","DOI":"10.1016\/j.lpm.2014.03.027","volume":"43","author":"V L\u00f3r\u00e1nd","year":"2014","unstructured":"L\u00f3r\u00e1nd V, Czirj\u00e1k L, Minier T. Musculoskeletal involvement in systemic sclerosis. Presse Med. 2014;43(10 Pt 2):e315\u2013328. https:\/\/doi.org\/10.1016\/j.lpm.2014.03.027.","journal-title":"Presse Med"},{"issue":"10","key":"392_CR93","doi-asserted-by":"publisher","first-page":"1743","DOI":"10.1007\/s00296-021-04956-7","volume":"41","author":"RV Karalilova","year":"2021","unstructured":"Karalilova RV, Batalov ZA, Sapundzhieva TL, Matucci-Cerinic M, Batalov AZ. Tofacitinib in the treatment of skin and musculoskeletal involvement in patients with systemic sclerosis, evaluated by ultrasound. Rheumatol Int. 2021;41(10):1743\u201353. https:\/\/doi.org\/10.1007\/s00296-021-04956-7.","journal-title":"Rheumatol Int"},{"key":"392_CR94","doi-asserted-by":"publisher","first-page":"974078","DOI":"10.3389\/fimmu.2022.974078","volume":"13","author":"M Giannini","year":"2023","unstructured":"Giannini M, Ellezam B, Leclair V, Lefebvre F, Troyanov Y, Hudson M, et al. Scleromyositis: a distinct novel entity within the systemic sclerosis and autoimmune myositis spectrum. Implications for care and pathogenesis. Front Immunol. 2023;13:974078. https:\/\/doi.org\/10.3389\/fimmu.2022.974078.","journal-title":"Front Immunol"},{"issue":"9","key":"392_CR95","doi-asserted-by":"publisher","first-page":"1613","DOI":"10.1002\/1529-0131(199809)41:9<1613::AID-ART11>3.0.CO;2-O","volume":"41","author":"VD Steen","year":"1998","unstructured":"Steen VD, Medsger TA Jr. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41(9):1613\u201319. https:\/\/doi.org\/10.1002\/1529-0131(199809)41:9<1613::AID-ART11>3.0.CO;2-O.","journal-title":"Arthritis Rheum"}],"container-title":["Advances in Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s42358-024-00392-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s42358-024-00392-w\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s42358-024-00392-w.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,10]],"date-time":"2024-07-10T06:14:07Z","timestamp":1720592047000},"score":1,"resource":{"primary":{"URL":"https:\/\/advancesinrheumatology.biomedcentral.com\/articles\/10.1186\/s42358-024-00392-w"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,7,10]]},"references-count":95,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["392"],"URL":"https:\/\/doi.org\/10.1186\/s42358-024-00392-w","relation":{},"ISSN":["2523-3106"],"issn-type":[{"value":"2523-3106","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,7,10]]},"assertion":[{"value":"30 September 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 June 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 July 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"52"}}