{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T18:19:09Z","timestamp":1789755549893,"version":"4.0.1"},"reference-count":159,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2022,3,7]],"date-time":"2022-03-07T00:00:00Z","timestamp":1646611200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,3,7]],"date-time":"2022-03-07T00:00:00Z","timestamp":1646611200000},"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":["Clinic Rev Allerg Immunol"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Systemic sclerosis (SSc) is a rare systemic autoimmune disease, characterized by the presence of three main actors: vasculopathy, immune activation, and fibrosis. This pathologic process is then translated in a clinical picture with great variability among different patients in terms of type of organ involvement, disease severity and prognosis. This heterogeneity is a main feature of SSc, which, in addition to the presence of early phases of the disease characterized by mild symptoms, can explain the high difficulty in establishing classification criteria, and in defining patients\u2019 subsets and disease outcomes. The definition of disease outcomes is particularly relevant in the setting of clinical trials, where the aim is to provide reliable endpoints, able to measure the magnitude of the efficacy of a certain drug or intervention. For this reason, in the last years, increasing efforts have been done to design measures of disease activity, damage, severity, and response to treatment, often in the context of composite indexes. When considering disease outcomes, the experience of the patient represents a relevant and complementary aspect. The tools able to capture this experience, the patient-reported outcomes, have been increasingly used in the last years in clinical practice and in clinical trials, both as primary and secondary endpoints. This comprehensive narrative review on SSc will therefore cover pathogenetic and histopathologic aspects, epidemiology, classification systems, and disease outcome measures, in order to focus on issues that are relevant for clinical research and design of clinical trials.<\/jats:p>","DOI":"10.1007\/s12016-022-08929-x","type":"journal-article","created":{"date-parts":[[2022,3,7]],"date-time":"2022-03-07T07:02:39Z","timestamp":1646636559000},"page":"358-377","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["A Narrative Review of Pathogenetic and Histopathologic Aspects, Epidemiology, Classification Systems, and Disease Outcome Measures in Systemic Sclerosis"],"prefix":"10.1007","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1860-6866","authenticated-orcid":false,"given":"Maria-Grazia","family":"Lazzaroni","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0913-0197","authenticated-orcid":false,"given":"Silvia","family":"Piantoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5990-2979","authenticated-orcid":false,"given":"Fabrizio","family":"Angeli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5798-8254","authenticated-orcid":false,"given":"Stefania","family":"Bertocchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3678-6124","authenticated-orcid":false,"given":"Franco","family":"Franceschini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5241-1918","authenticated-orcid":false,"given":"Paolo","family":"Air\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,3,7]]},"reference":[{"key":"8929_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 (2017) Systemic sclerosis. Lancet 390:1685\u20131699. https:\/\/doi.org\/10.1016\/S0140-6736(17)30933-9","journal-title":"Lancet"},{"key":"8929_CR2","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 et al (2011) 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 70:476\u2013481. https:\/\/doi.org\/10.1136\/ard.2010.136929","journal-title":"Ann Rheum Dis"},{"key":"8929_CR3","doi-asserted-by":"publisher","first-page":"S3","DOI":"10.1016\/j.semarthrit.2019.09.019","volume":"49","author":"CP Denton","year":"2019","unstructured":"Denton CP (2019) Challenges in systemic sclerosis trial design. Semin Arthritis Rheum 49:S3\u2013S7. https:\/\/doi.org\/10.1016\/j.semarthrit.2019.09.019","journal-title":"Semin Arthritis Rheum"},{"key":"8929_CR4","doi-asserted-by":"publisher","first-page":"E2687","DOI":"10.3390\/jcm9092687","volume":"9","author":"Y Asano","year":"2020","unstructured":"Asano Y (2020) The pathogenesis of systemic sclerosis: an understanding based on a common pathologic cascade across multiple organs and additional organ-specific pathologies. J Clin Med 9:E2687. https:\/\/doi.org\/10.3390\/jcm9092687","journal-title":"J Clin Med"},{"key":"8929_CR5","doi-asserted-by":"publisher","first-page":"1300","DOI":"10.1002\/art.38326","volume":"66","author":"M Kuwana","year":"2014","unstructured":"Kuwana M, Okazaki Y (2014) Brief report: impaired in vivo neovascularization capacity of endothelial progenitor cells in patients with systemic sclerosis. Arthritis Rheumatol 66:1300\u20131305. https:\/\/doi.org\/10.1002\/art.38326","journal-title":"Arthritis Rheumatol"},{"key":"8929_CR6","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1684\/ecn.2013.0325","volume":"23","author":"NC Brembilla","year":"2012","unstructured":"Brembilla NC, Chizzolini C (2012) T cell abnormalities in systemic sclerosis with a focus on Th17 cells. Eur Cytokine Netw 23:128\u2013139. https:\/\/doi.org\/10.1684\/ecn.2013.0325","journal-title":"Eur Cytokine Netw"},{"key":"8929_CR7","doi-asserted-by":"publisher","first-page":"R4","DOI":"10.1186\/ar4430","volume":"16","author":"X Yang","year":"2014","unstructured":"Yang X, Yang J, Xing X et al (2014) Increased frequency of Th17 cells in systemic sclerosis is related to disease activity and collagen overproduction. Arthritis Res Ther 16:R4. https:\/\/doi.org\/10.1186\/ar4430","journal-title":"Arthritis Res Ther"},{"key":"8929_CR8","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/835948","volume":"2013","author":"SA Jimenez","year":"2013","unstructured":"Jimenez SA (2013) Role of endothelial to mesenchymal transition in the pathogenesis of the vascular alterations in systemic sclerosis. ISRN Rheumatol 2013:835948. https:\/\/doi.org\/10.1155\/2013\/835948","journal-title":"ISRN Rheumatol"},{"key":"8929_CR9","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1093\/rheumatology\/kes315","volume":"52","author":"D Schneeberger","year":"2013","unstructured":"Schneeberger D, Tyndall A, Kay J et al (2013) Systemic sclerosis without antinuclear antibodies or Raynaud\u2019s phenomenon: a multicentre study in the prospective EULAR Scleroderma Trials and Research (EUSTAR) database. Rheumatology (Oxford) 52:560\u2013567. https:\/\/doi.org\/10.1093\/rheumatology\/kes315","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR10","doi-asserted-by":"publisher","first-page":"3317","DOI":"10.1007\/s10067-019-04745-5","volume":"38","author":"JD Pauling","year":"2019","unstructured":"Pauling JD, Hughes M, Pope JE (2019) Raynaud\u2019s phenomenon-an update on diagnosis, classification and management. Clin Rheumatol 38:3317\u20133330. https:\/\/doi.org\/10.1007\/s10067-019-04745-5","journal-title":"Clin Rheumatol"},{"key":"8929_CR11","doi-asserted-by":"publisher","first-page":"1119","DOI":"10.1002\/acr.23729","volume":"71","author":"JD Pauling","year":"2019","unstructured":"Pauling JD, Reilly E, Smith T, Frech TM (2019) Evolving symptom characteristics of Raynaud\u2019s phenomenon in systemic sclerosis and their association with physician and patient-reported assessments of disease severity. Arthritis Care Res (Hoboken) 71:1119\u20131126. https:\/\/doi.org\/10.1002\/acr.23729","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"8929_CR12","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1093\/rheumatology\/kem085","volume":"46","author":"H Gunawardena","year":"2007","unstructured":"Gunawardena H, Harris ND, Carmichael C, McHugh NJ (2007) Maximum blood flow and microvascular regulatory responses in systemic sclerosis. Rheumatology (Oxford) 46:1079\u20131082. https:\/\/doi.org\/10.1093\/rheumatology\/kem085","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR13","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1006\/niox.2000.0309","volume":"4","author":"A Sud","year":"2000","unstructured":"Sud A, Khullar M, Wanchu A, Bambery P (2000) Increased nitric oxide production in patients with systemic sclerosis. Nitric Oxide 4:615\u2013619. https:\/\/doi.org\/10.1006\/niox.2000.0309","journal-title":"Nitric Oxide"},{"key":"8929_CR14","doi-asserted-by":"publisher","first-page":"221","DOI":"10.1016\/s0098-2997(99)00002-3","volume":"19","author":"JP Eiserich","year":"1998","unstructured":"Eiserich JP, Patel RP, O\u2019Donnell VB (1998) Pathophysiology of nitric oxide and related species: free radical reactions and modification of biomolecules. Mol Aspects Med 19:221\u2013357. https:\/\/doi.org\/10.1016\/s0098-2997(99)00002-3","journal-title":"Mol Aspects Med"},{"key":"8929_CR15","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1002\/(SICI)1096-9896(199910)189:2<273::AID-PATH413>3.0.CO;2-4","volume":"189","author":"SA Cotton","year":"1999","unstructured":"Cotton SA, Herrick AL, Jayson MI, Freemont AJ (1999) Endothelial expression of nitric oxide synthases and nitrotyrosine in systemic sclerosis skin. J Pathol 189:273\u2013278. https:\/\/doi.org\/10.1002\/(SICI)1096-9896(199910)189:2%3c273::AID-PATH413%3e3.0.CO;2-4","journal-title":"J Pathol"},{"key":"8929_CR16","doi-asserted-by":"publisher","first-page":"1324","DOI":"10.1002\/art.10191","volume":"46","author":"GN Andersen","year":"2002","unstructured":"Andersen GN, Mincheva-Nilsson L, Kazzam E et al (2002) Assessment of vascular function in systemic sclerosis: indications of the development of nitrate tolerance as a result of enhanced endothelial nitric oxide production. Arthritis Rheum 46:1324\u20131332. https:\/\/doi.org\/10.1002\/art.10191","journal-title":"Arthritis Rheum"},{"key":"8929_CR17","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1002\/art.21543","volume":"54","author":"L Ibba-Manneschi","year":"2006","unstructured":"Ibba-Manneschi L, Niissalo S, Milia AF et al (2006) Variations of neuronal nitric oxide synthase in systemic sclerosis skin. Arthritis Rheum 54:202\u2013213. https:\/\/doi.org\/10.1002\/art.21543","journal-title":"Arthritis Rheum"},{"key":"8929_CR18","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1097\/00005792-198011000-00001","volume":"59","author":"GP Rodnan","year":"1980","unstructured":"Rodnan GP, Myerowitz RL, Justh GO (1980) Morphologic changes in the digital arteries of patients with progressive systemic sclerosis (scleroderma) and Raynaud phenomenon. Medicine (Baltimore) 59:393\u2013408. https:\/\/doi.org\/10.1097\/00005792-198011000-00001","journal-title":"Medicine (Baltimore)"},{"key":"8929_CR19","doi-asserted-by":"publisher","unstructured":"Distler JHW, Gay S, Distler O (2006) Angiogenesis and vasculogenesis in systemic sclerosis. Rheumatology (Oxford) 45(Suppl_3):iii26\u2013iii27. https:\/\/doi.org\/10.1093\/rheumatology\/kel295","DOI":"10.1093\/rheumatology\/kel295"},{"key":"8929_CR20","doi-asserted-by":"publisher","first-page":"e14","DOI":"10.1161\/CIRCRESAHA.111.242057","volume":"109","author":"M Manetti","year":"2011","unstructured":"Manetti M, Guiducci S, Romano E et al (2011) Overexpression of VEGF165b, an inhibitory splice variant of vascular endothelial growth factor, leads to insufficient angiogenesis in patients with systemic sclerosis. Circ Res 109:e14-26. https:\/\/doi.org\/10.1161\/CIRCRESAHA.111.242057","journal-title":"Circ Res"},{"key":"8929_CR21","doi-asserted-by":"publisher","first-page":"1886","DOI":"10.1002\/1529-0131(200008)43:8<1886::AID-ANR27>3.0.CO;2-S","volume":"43","author":"NA Flavahan","year":"2000","unstructured":"Flavahan NA, Flavahan S, Liu Q et al (2000) Increased alpha2-adrenergic constriction of isolated arterioles in diffuse scleroderma. Arthritis Rheum 43:1886\u20131890. https:\/\/doi.org\/10.1002\/1529-0131(200008)43:8%3c1886::AID-ANR27%3e3.0.CO;2-S","journal-title":"Arthritis Rheum"},{"key":"8929_CR22","doi-asserted-by":"publisher","first-page":"488","DOI":"10.1093\/rheumatology\/keh517","volume":"44","author":"PB Furspan","year":"2005","unstructured":"Furspan PB, Chatterjee S, Mayes MD, Freedman RR (2005) Cooling-induced contraction and protein tyrosine kinase activity of isolated arterioles in secondary Raynaud\u2019s phenomenon. Rheumatology (Oxford) 44:488\u2013494. https:\/\/doi.org\/10.1093\/rheumatology\/keh517","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR23","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1038\/nrrheum.2012.96","volume":"8","author":"AL Herrick","year":"2012","unstructured":"Herrick AL (2012) The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol 8:469\u2013479. https:\/\/doi.org\/10.1038\/nrrheum.2012.96","journal-title":"Nat Rev Rheumatol"},{"key":"8929_CR24","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1007\/s00281-008-0125-4","volume":"30","author":"A Gabrielli","year":"2008","unstructured":"Gabrielli A, Svegliati S, Moroncini G et al (2008) Oxidative stress and the pathogenesis of scleroderma: the Murrell\u2019s hypothesis revisited. Semin Immunopathol 30:329\u2013337. https:\/\/doi.org\/10.1007\/s00281-008-0125-4","journal-title":"Semin Immunopathol"},{"key":"8929_CR25","doi-asserted-by":"publisher","first-page":"1285","DOI":"10.1136\/annrheumdis-2015-207271","volume":"75","author":"EG Wirz","year":"2016","unstructured":"Wirz EG, Jaeger VK, Allanore Y et al (2016) Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: a 10-year longitudinal study from the EUSTAR database. Ann Rheum Dis 75:1285\u20131292. https:\/\/doi.org\/10.1136\/annrheumdis-2015-207271","journal-title":"Ann Rheum Dis"},{"key":"8929_CR26","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1136\/annrheumdis-2011-200631","volume":"71","author":"CP Denton","year":"2012","unstructured":"Denton CP, Krieg T, Guillevin L et al (2012) Demographic, clinical and antibody characteristics of patients with digital ulcers in systemic sclerosis: data from the DUO Registry. Ann Rheum Dis 71:718\u2013721. https:\/\/doi.org\/10.1136\/annrheumdis-2011-200631","journal-title":"Ann Rheum Dis"},{"key":"8929_CR27","doi-asserted-by":"publisher","unstructured":"Steen V, Denton CP, Pope JE, Matucci-Cerinic M (2009) Digital ulcers: overt vascular disease in systemic sclerosis. Rheumatology (Oxford) 48(Suppl_3):iii19\u2013iii24. https:\/\/doi.org\/10.1093\/rheumatology\/kep105","DOI":"10.1093\/rheumatology\/kep105"},{"key":"8929_CR28","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1093\/rheumatology\/keu296","volume":"54","author":"C Bruni","year":"2015","unstructured":"Bruni C, Guiducci S, Bellando-Randone S et al (2015) Digital ulcers as a sentinel sign for early internal organ involvement in very early systemic sclerosis. Rheumatology (Oxford) 54:72\u201376. https:\/\/doi.org\/10.1093\/rheumatology\/keu296","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR29","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1093\/rheumatology\/ker414","volume":"51","author":"M Frerix","year":"2012","unstructured":"Frerix M, Stegbauer J, Dragun D et al (2012) Ulnar artery occlusion is predictive of digital ulcers in SSc: a duplex sonography study. Rheumatology (Oxford) 51:735\u2013742. https:\/\/doi.org\/10.1093\/rheumatology\/ker414","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR30","doi-asserted-by":"publisher","first-page":"1374","DOI":"10.1093\/rheumatology\/keq097","volume":"49","author":"L Amanzi","year":"2010","unstructured":"Amanzi L, Braschi F, Fiori G et al (2010) Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions. Rheumatology (Oxford) 49:1374\u20131382. https:\/\/doi.org\/10.1093\/rheumatology\/keq097","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR31","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1007\/s10067-012-2161-7","volume":"32","author":"D Giuggioli","year":"2013","unstructured":"Giuggioli D, Manfredi A, Colaci M et al (2013) Osteomyelitis complicating scleroderma digital ulcers. Clin Rheumatol 32:623\u2013627. https:\/\/doi.org\/10.1007\/s10067-012-2161-7","journal-title":"Clin Rheumatol"},{"key":"8929_CR32","doi-asserted-by":"publisher","DOI":"10.5152\/eurjrheum.2020.20048","author":"E Zanatta","year":"2020","unstructured":"Zanatta E, Codullo V, Allanore Y (2020) Scleroderma renal crisis: case reports and update on critical issues. Eur J Rheumatol. https:\/\/doi.org\/10.5152\/eurjrheum.2020.20048","journal-title":"Eur J Rheumatol"},{"key":"8929_CR33","doi-asserted-by":"publisher","first-page":"1625","DOI":"10.1002\/art.38390","volume":"66","author":"SI Nihtyanova","year":"2014","unstructured":"Nihtyanova SI, Schreiber BE, Ong VH et al (2014) Prediction of pulmonary complications and long-term survival in systemic sclerosis. Arthritis Rheumatol 66:1625\u20131635. https:\/\/doi.org\/10.1002\/art.38390","journal-title":"Arthritis Rheumatol"},{"key":"8929_CR34","doi-asserted-by":"publisher","first-page":"639","DOI":"10.3899\/jrheum.160817","volume":"44","author":"M-G Lazzaroni","year":"2017","unstructured":"Lazzaroni M-G, Cavazzana I, Colombo E et al (2017) Malignancies in patients with anti-RNA polymerase III antibodies and systemic sclerosis: analysis of the EULAR scleroderma trials and research cohort and possible recommendations for screening. J Rheumatol 44:639\u2013647. https:\/\/doi.org\/10.3899\/jrheum.160817","journal-title":"J Rheumatol"},{"key":"8929_CR35","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1177\/2397198318786158","volume":"3","author":"M-G Lazzaroni","year":"2018","unstructured":"Lazzaroni M-G, Air\u00f2 P (2018) Anti-RNA polymerase III antibodies in patients with suspected and definite systemic sclerosis: why and how to screen. J Scleroderma Relat Disord 3:214\u2013220. https:\/\/doi.org\/10.1177\/2397198318786158","journal-title":"Journal of Scleroderma and Related Disorders"},{"key":"8929_CR36","doi-asserted-by":"publisher","first-page":"1829","DOI":"10.3899\/jrheum.111417","volume":"39","author":"M Caron","year":"2012","unstructured":"Caron M, Hudson M, Baron M et al (2012) Longitudinal study of renal function in systemic sclerosis. J Rheumatol 39:1829\u20131834. https:\/\/doi.org\/10.3899\/jrheum.111417","journal-title":"J Rheumatol"},{"key":"8929_CR37","doi-asserted-by":"publisher","DOI":"10.1155\/2010\/543704","volume":"2010","author":"I Batal","year":"2010","unstructured":"Batal I, Domsic RT, Medsger TA, Bastacky S (2010) Scleroderma renal crisis: a pathology perspective. Int J Rheumatol 2010:543704. https:\/\/doi.org\/10.1155\/2010\/543704","journal-title":"Int J Rheumatol"},{"key":"8929_CR38","doi-asserted-by":"publisher","first-page":"332","DOI":"10.1016\/j.humpath.2008.08.001","volume":"40","author":"I Batal","year":"2009","unstructured":"Batal I, Domsic RT, Shafer A et al (2009) Renal biopsy findings predicting outcome in scleroderma renal crisis. Hum Pathol 40:332\u2013340. https:\/\/doi.org\/10.1016\/j.humpath.2008.08.001","journal-title":"Hum Pathol"},{"key":"8929_CR39","doi-asserted-by":"publisher","first-page":"D42","DOI":"10.1016\/j.jacc.2013.10.032","volume":"62","author":"MM Hoeper","year":"2013","unstructured":"Hoeper MM, Bogaard HJ, Condliffe R et al (2013) Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62:D42-50. https:\/\/doi.org\/10.1016\/j.jacc.2013.10.032","journal-title":"J Am Coll Cardiol"},{"key":"8929_CR40","doi-asserted-by":"publisher","first-page":"1346","DOI":"10.1378\/chest.12-2396","volume":"144","author":"NF Chaisson","year":"2013","unstructured":"Chaisson NF, Hassoun PM (2013) Systemic sclerosis-associated pulmonary arterial hypertension. Chest 144:1346\u20131356. https:\/\/doi.org\/10.1378\/chest.12-2396","journal-title":"Chest"},{"key":"8929_CR41","doi-asserted-by":"publisher","first-page":"3043","DOI":"10.1002\/art.22069","volume":"54","author":"MR Fisher","year":"2006","unstructured":"Fisher MR, Mathai SC, Champion HC et al (2006) Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 54:3043\u20133050. https:\/\/doi.org\/10.1002\/art.22069","journal-title":"Arthritis Rheum"},{"key":"8929_CR42","doi-asserted-by":"publisher","first-page":"1494","DOI":"10.1378\/chest.13-3014","volume":"146","author":"L Chung","year":"2014","unstructured":"Chung L, Farber HW, Benza R et al (2014) Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL registry. Chest 146:1494\u20131504. https:\/\/doi.org\/10.1378\/chest.13-3014","journal-title":"Chest"},{"key":"8929_CR43","doi-asserted-by":"publisher","first-page":"2493","DOI":"10.1093\/eurheartj\/ehp297","volume":"30","author":"N Gali\u00e8","year":"2009","unstructured":"Gali\u00e8 N, Hoeper MM, Humbert M et al (2009) Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 30:2493\u20132537. https:\/\/doi.org\/10.1093\/eurheartj\/ehp297","journal-title":"Eur Heart J"},{"key":"8929_CR44","doi-asserted-by":"publisher","first-page":"2360","DOI":"10.1161\/CIRCULATIONAHA.117.033147","volume":"137","author":"S Hsu","year":"2018","unstructured":"Hsu S, Kokkonen-Simon KM, Kirk JA et al (2018) Right ventricular myofilament functional differences in humans with systemic sclerosis-associated versus idiopathic pulmonary arterial hypertension. Circulation 137:2360\u20132370. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.117.033147","journal-title":"Circulation"},{"key":"8929_CR45","doi-asserted-by":"publisher","first-page":"581","DOI":"10.1002\/art.1780230510","volume":"23","author":"AT Masi","year":"1980","unstructured":"Masi AT (1980) Preliminary criteria for the classification of systemic sclerosis (scleroderma). Arthritis Rheum 23:581\u2013590. https:\/\/doi.org\/10.1002\/art.1780230510","journal-title":"Arthritis Rheum"},{"key":"8929_CR46","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1002\/1529-0131(200103)44:3<735::AID-ANR125>3.0.CO;2-F","volume":"44","author":"LS Lonzetti","year":"2001","unstructured":"Lonzetti LS, Joyal F, Raynauld J-P et al (2001) Updating the American College of Rheumatology preliminary classification criteria for systemic sclerosis: addition of severe nailfold capillaroscopy abnormalities markedly increases the sensitivity for limited scleroderma. Arthritis Rheum 44:735\u2013736. https:\/\/doi.org\/10.1002\/1529-0131(200103)44:3%3c735::AID-ANR125%3e3.0.CO;2-F","journal-title":"Arthritis Rheum"},{"key":"8929_CR47","first-page":"1573","volume":"28","author":"EC LeRoy","year":"2001","unstructured":"LeRoy EC, Medsger TA (2001) Criteria for the classification of early systemic sclerosis. J Rheumatol 28:1573\u20131576","journal-title":"J Rheumatol"},{"key":"8929_CR48","first-page":"1471","volume":"28","author":"FM Wigley","year":"2001","unstructured":"Wigley FM (2001) When is scleroderma really scleroderma? J Rheumatol 28:1471\u20131473","journal-title":"J Rheumatol"},{"key":"8929_CR49","doi-asserted-by":"publisher","first-page":"3902","DOI":"10.1002\/art.24038","volume":"58","author":"M Koenig","year":"2008","unstructured":"Koenig M, Joyal F, Fritzler MJ et al (2008) Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud\u2019s phenomenon to systemic sclerosis: a twenty-year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis. Arthritis Rheum 58:3902\u20133912. https:\/\/doi.org\/10.1002\/art.24038","journal-title":"Arthritis Rheum"},{"key":"8929_CR50","doi-asserted-by":"publisher","first-page":"2087","DOI":"10.1136\/annrheumdis-2013-203716","volume":"73","author":"T Minier","year":"2014","unstructured":"Minier T, Guiducci S, Bellando-Randone S et al (2014) Preliminary analysis of the Very Early Diagnosis of Systemic Sclerosis (VEDOSS) EUSTAR multicentre study: evidence for puffy fingers as a pivotal sign for suspicion of systemic sclerosis. Ann Rheum Dis 73:2087\u20132093. https:\/\/doi.org\/10.1136\/annrheumdis-2013-203716","journal-title":"Ann Rheum Dis"},{"key":"8929_CR51","doi-asserted-by":"publisher","first-page":"2737","DOI":"10.1002\/art.38098","volume":"65","author":"F van den Hoogen","year":"2013","unstructured":"van den Hoogen F, Khanna D, Fransen J et al (2013) 2013 Classification criteria for systemic sclerosis: an American College of Rheumatology\/European League Against Rheumatism collaborative initiative. Arthritis Rheum 65:2737\u20132747. https:\/\/doi.org\/10.1002\/art.38098","journal-title":"Arthritis Rheum"},{"key":"8929_CR52","doi-asserted-by":"publisher","first-page":"8","DOI":"10.3899\/jrheum.141020","volume":"42","author":"AT Masi","year":"2015","unstructured":"Masi AT, Medsger TA (2015) Progress in the evolution of systemic sclerosis classification criteria and recommendation for additional comparative specificity studies. J Rheumatol 42:8\u201310. https:\/\/doi.org\/10.3899\/jrheum.141020","journal-title":"J Rheumatol"},{"key":"8929_CR53","doi-asserted-by":"publisher","first-page":"350","DOI":"10.1016\/j.semarthrit.2016.05.007","volume":"46","author":"S Melchor","year":"2016","unstructured":"Melchor S, Joven BE, Andreu JL et al (2016) Validation of the 2013 American College of Rheumatology\/European League Against Rheumatism classification criteria for systemic sclerosis in patients from a capillaroscopy clinic. Semin Arthritis Rheum 46:350\u2013355. https:\/\/doi.org\/10.1016\/j.semarthrit.2016.05.007","journal-title":"Semin Arthritis Rheum"},{"key":"8929_CR54","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0166629","volume":"11","author":"D Xu","year":"2016","unstructured":"Xu D, Hou Y, Zheng Y et al (2016) The 2013 American College of Rheumatology\/European League Against Rheumatism classification criteria for systemic sclerosis could classify systemic sclerosis patients at earlier stage: data from a Chinese EUSTAR center. PLoS One 11:e0166629. https:\/\/doi.org\/10.1371\/journal.pone.0166629","journal-title":"PLoS ONE"},{"key":"8929_CR55","doi-asserted-by":"publisher","first-page":"60","DOI":"10.3899\/jrheum.140047","volume":"42","author":"A-M Hoffmann-Vold","year":"2015","unstructured":"Hoffmann-Vold A-M, Gunnarsson R, Garen T et al (2015) Performance of the 2013 American College of Rheumatology\/European League Against Rheumatism classification criteria for systemic sclerosis (SSc) in large, well-defined cohorts of ssc and mixed connective tissue disease. J Rheumatol 42:60\u201363. https:\/\/doi.org\/10.3899\/jrheum.140047","journal-title":"J Rheumatol"},{"key":"8929_CR56","first-page":"1531","volume":"41","author":"H Alhajeri","year":"2014","unstructured":"Alhajeri H, Hudson M, Fritzler M et al (2014) Evaluation of the new ACR\/EULAR criteria for the classification of systemic sclerosis in the Canadian Scleroderma Research Group cohort. J Rheumatol 41:1531\u20131532","journal-title":"J Rheumatol"},{"key":"8929_CR57","doi-asserted-by":"publisher","first-page":"1454","DOI":"10.1093\/rheumatology\/keu530","volume":"54","author":"S Jordan","year":"2015","unstructured":"Jordan S, Maurer B, Toniolo M et al (2015) Performance of the new ACR\/EULAR classification criteria for systemic sclerosis in clinical practice. Rheumatology 54:1454\u20131458. https:\/\/doi.org\/10.1093\/rheumatology\/keu530","journal-title":"Rheumatology"},{"key":"8929_CR58","doi-asserted-by":"publisher","first-page":"614","DOI":"10.1080\/14397595.2016.1250332","volume":"27","author":"Y Ikawa","year":"2017","unstructured":"Ikawa Y, Hamaguchi Y, Mugii N et al (2017) Classification of Japanese patients with mild\/early systemic sclerosis (SSc) by the 2013 ACR\/EULAR classification criteria for SSc. Mod Rheumatol 27:614\u2013617. https:\/\/doi.org\/10.1080\/14397595.2016.1250332","journal-title":"Mod Rheumatol"},{"key":"8929_CR59","doi-asserted-by":"publisher","first-page":"370","DOI":"10.1007\/s10067-004-1049-6","volume":"24","author":"P Air\u00f2","year":"2005","unstructured":"Air\u00f2 P, Della Casa D, Danieli E et al (2005) Oesophageal manometry in early and definite systemic sclerosis. Clin Rheumatol 24:370\u2013376. https:\/\/doi.org\/10.1007\/s10067-004-1049-6","journal-title":"Clin Rheumatol"},{"key":"8929_CR60","doi-asserted-by":"publisher","first-page":"317","DOI":"10.1093\/rheumatology\/keq176","volume":"50","author":"G Valentini","year":"2011","unstructured":"Valentini G, Cuomo G, Abignano G et al (2011) Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features. Rheumatology 50:317\u2013323. https:\/\/doi.org\/10.1093\/rheumatology\/keq176","journal-title":"Rheumatology"},{"key":"8929_CR61","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2020.102659","volume":"19","author":"G Valentini","year":"2020","unstructured":"Valentini G, Pope JE (2020) Undifferentiated connective tissue disease at risk for systemic sclerosis: which patients might be labeled prescleroderma? Autoimmun Rev 19:102659. https:\/\/doi.org\/10.1016\/j.autrev.2020.102659","journal-title":"Autoimmun Rev"},{"key":"8929_CR62","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2021.102751","volume":"20","author":"A Riccardi","year":"2021","unstructured":"Riccardi A, Marcoccia A, Modesti M et al (2021) Undifferentiated connective tissue disease at risk for systemic sclerosis: development of a short-term predictive score and a risk stratification tool. Autoimmun Rev 20:102751. https:\/\/doi.org\/10.1016\/j.autrev.2021.102751","journal-title":"Autoimmun Rev"},{"key":"8929_CR63","doi-asserted-by":"publisher","first-page":"11","DOI":"10.3899\/jrheum.141103","volume":"42","author":"JE Pope","year":"2015","unstructured":"Pope JE (2015) Systemic sclerosis classification: a rose by any other name would smell as sweet? J Rheumatol 42:11\u201313. https:\/\/doi.org\/10.3899\/jrheum.141103","journal-title":"J Rheumatol"},{"key":"8929_CR64","first-page":"202","volume":"15","author":"EC LeRoy","year":"1988","unstructured":"LeRoy EC, Black C, Fleischmajer R et al (1988) Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 15:202\u2013205","journal-title":"J Rheumatol"},{"key":"8929_CR65","first-page":"911","volume":"13","author":"M Giordano","year":"1986","unstructured":"Giordano M, Valentini G, Migliaresi S et al (1986) Different antibody patterns and different prognoses in patients with scleroderma with various extent of skin sclerosis. J Rheumatol 13:911\u2013916","journal-title":"J Rheumatol"},{"key":"8929_CR66","first-page":"276","volume":"15","author":"AJ Barnett","year":"1988","unstructured":"Barnett AJ, Miller MH, Littlejohn GO (1988) A survival study of patients with scleroderma diagnosed over 30 years (1953\u20131983): the value of a simple cutaneous classification in the early stages of the disease. J Rheumatol 15:276\u2013283","journal-title":"J Rheumatol"},{"key":"8929_CR67","doi-asserted-by":"publisher","first-page":"154","DOI":"10.1097\/00005792-200203000-00005","volume":"81","author":"L Scussel-Lonzetti","year":"2002","unstructured":"Scussel-Lonzetti L, Joyal F, Raynauld J-P et al (2002) Predicting mortality in systemic sclerosis: analysis of a cohort of 309 French Canadian patients with emphasis on features at diagnosis as predictive factors for survival. Medicine 81:154\u2013167","journal-title":"Medicine"},{"key":"8929_CR68","doi-asserted-by":"publisher","first-page":"1553","DOI":"10.1002\/art.40906","volume":"71","author":"V Sobanski","year":"2019","unstructured":"Sobanski V, Giovannelli J, Allanore Y et al (2019) Phenotypes determined by cluster analysis and their survival in the prospective european scleroderma trials and research cohort of patients with systemic sclerosis. Arthritis Rheumatol 71:1553\u20131570. https:\/\/doi.org\/10.1002\/art.40906","journal-title":"Arthritis & Rheumatology"},{"issue":"Suppl 113","key":"8929_CR69","first-page":"109","volume":"36","author":"M Boonstra","year":"2018","unstructured":"Boonstra M, Mertens BJA, Bakker JA et al (2018) To what extent do autoantibodies help to identify high-risk patients in systemic sclerosis? Clin Exp Rheumatol 36(Suppl_113):109\u2013117","journal-title":"Clin Exp Rheumatol"},{"key":"8929_CR70","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.201594","author":"T Nevskaya","year":"2021","unstructured":"Nevskaya T, Pope JE, Turk MA et al (2021) Systematic analysis of the literature in search of defining systemic sclerosis subsets. J Rheumatol. https:\/\/doi.org\/10.3899\/jrheum.201594","journal-title":"J Rheumatol"},{"key":"8929_CR71","doi-asserted-by":"publisher","first-page":"386","DOI":"10.1136\/annrheumdis-2021-eular.3048","volume":"80","author":"E Zanatta","year":"2021","unstructured":"Zanatta E, Huscher D, Air\u00f2 P et al (2021) Clinical phenotype in scleroderma patients with anti-topoisomerase I positivity and limited cutaneous form: data from the EUSTAR database. Ann Rheum Dis 80:386\u2013387. https:\/\/doi.org\/10.1136\/annrheumdis-2021-eular.3048","journal-title":"Ann Rheum Dis"},{"key":"8929_CR72","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1016\/j.semarthrit.2015.04.009","volume":"45","author":"N Srivastava","year":"2015","unstructured":"Srivastava N, Hudson M, Tatibouet S et al (2015) Thinking outside the box\u2014the associations with cutaneous involvement and autoantibody status in systemic sclerosis are not always what we expect. Semin Arthritis Rheum 45:184\u2013189. https:\/\/doi.org\/10.1016\/j.semarthrit.2015.04.009","journal-title":"Semin Arthritis Rheum"},{"key":"8929_CR73","doi-asserted-by":"publisher","first-page":"465","DOI":"10.1002\/art.41153","volume":"72","author":"SI Nihtyanova","year":"2020","unstructured":"Nihtyanova SI, Sari A, Harvey JC et al (2020) Using autoantibodies and cutaneous subset to develop outcome-based disease classification in systemic sclerosis. Arthritis Rheumatol 72:465\u2013476. https:\/\/doi.org\/10.1002\/art.41153","journal-title":"Arthritis & Rheumatology"},{"key":"8929_CR74","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1080\/13543784.2021.1923693","volume":"1","author":"A Lescoat","year":"2021","unstructured":"Lescoat A, Varga J, Matucci-Cerinic M, Khanna D (2021) New promising drugs for the treatment of systemic sclerosis: pathogenic considerations, enhanced classifications, and personalized medicine. Expert Opin Investig Drugs 0:1\u201318. https:\/\/doi.org\/10.1080\/13543784.2021.1923693","journal-title":"Expert Opin Investig Drugs"},{"key":"8929_CR75","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2020.102515","volume":"19","author":"RL Smeets","year":"2020","unstructured":"Smeets RL, Kersten BE, Joosten I et al (2020) Diagnostic profiles for precision medicine in systemic sclerosis; stepping forward from single biomarkers towards pathophysiological panels. Autoimmun Rev 19:102515. https:\/\/doi.org\/10.1016\/j.autrev.2020.102515","journal-title":"Autoimmun Rev"},{"key":"8929_CR76","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1097\/BOR.0000000000000785","volume":"33","author":"LM Calderon","year":"2021","unstructured":"Calderon LM, Pope JE (2021) Scleroderma epidemiology update. Curr Opin Rheumatol 33:122\u2013127. https:\/\/doi.org\/10.1097\/BOR.0000000000000785","journal-title":"Curr Opin Rheumatol"},{"key":"8929_CR77","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab190","author":"M Bairkdar","year":"2021","unstructured":"Bairkdar M, Rossides M, Westerlind H et al (2021) Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis. Rheumatology (Oxford). https:\/\/doi.org\/10.1093\/rheumatology\/keab190","journal-title":"Rheumatology (Oxford)"},{"key":"8929_CR78","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1016\/j.semarthrit.2007.05.003","volume":"37","author":"H Chifflot","year":"2008","unstructured":"Chifflot H, Fautrel B, Sordet C et al (2008) Incidence and prevalence of systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 37:223\u2013235. https:\/\/doi.org\/10.1016\/j.semarthrit.2007.05.003","journal-title":"Semin Arthritis Rheum"},{"key":"8929_CR79","doi-asserted-by":"publisher","first-page":"2096","DOI":"10.1111\/1756-185X.13716","volume":"22","author":"L Zhong","year":"2019","unstructured":"Zhong L, Pope M, Shen Y et al (2019) Prevalence and incidence of systemic sclerosis: a systematic review and meta-analysis. Int J Rheum Dis 22:2096\u20132107. https:\/\/doi.org\/10.1111\/1756-185X.13716","journal-title":"Int J Rheum Dis"},{"key":"8929_CR80","first-page":"S10","volume":"29","author":"A Lo Monaco","year":"2011","unstructured":"Lo Monaco A, Bruschi M, La Corte R et al (2011) Epidemiology of systemic sclerosis in a district of northern Italy. Clin Exp Rheumatol 29:S10-14","journal-title":"Clin Exp Rheumatol"},{"key":"8929_CR81","first-page":"878","volume":"25","author":"P Air\u00f2","year":"2007","unstructured":"Air\u00f2 P, Tabaglio E, Frassi M et al (2007) Prevalence of systemic sclerosis in Valtrompia in northern Italy. A collaborative study of rheumatologists and general practitioners. Clin Exp Rheumatol 25:878\u2013880","journal-title":"Clin Exp Rheumatol"},{"key":"8929_CR82","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1177\/2397198318819908","volume":"5","author":"P Air\u00f2","year":"2020","unstructured":"Air\u00f2 P, Regola F, Lazzaroni M-G et al (2020) Incidence and prevalence of systemic sclerosis in Valcamonica, Italy, during an 18-year period. J Scleroderma Relat Disord 5:51\u201356. https:\/\/doi.org\/10.1177\/2397198318819908","journal-title":"Journal of Scleroderma and Related Disorders"},{"key":"8929_CR83","doi-asserted-by":"publisher","first-page":"1788","DOI":"10.1136\/annrheumdis-2013-203618","volume":"73","author":"K Andr\u00e9asson","year":"2014","unstructured":"Andr\u00e9asson K, Saxne T, Bergknut C et al (2014) Prevalence and incidence of systemic sclerosis in southern Sweden: population-based data with case ascertainment using the 1980 ARA criteria and the proposed ACR-EULAR classification criteria. Ann Rheum Dis 73:1788\u20131792. https:\/\/doi.org\/10.1136\/annrheumdis-2013-203618","journal-title":"Ann Rheum Dis"},{"key":"8929_CR84","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1002\/art.1780400309","volume":"40","author":"VD Steen","year":"1997","unstructured":"Steen VD, Oddis CV, Conte CG et al (1997) Incidence of systemic sclerosis in allegheny county, Pennsylvania. A twenty-year study of hospital-diagnosed cases, 1963\u20131982. Arthritis Rheum 40:441\u2013445. https:\/\/doi.org\/10.1002\/art.1780400309","journal-title":"Arthritis Rheum"},{"key":"8929_CR85","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1111\/j.1445-5994.2006.01125.x","volume":"36","author":"PJ Roberts-Thomson","year":"2006","unstructured":"Roberts-Thomson PJ, Walker JG, Lu TY-T et al (2006) Scleroderma in South Australia: further epidemiological observations supporting a stochastic explanation. Intern Med J 36:489\u2013497. https:\/\/doi.org\/10.1111\/j.1445-5994.2006.01125.x","journal-title":"Intern Med J"},{"key":"8929_CR86","doi-asserted-by":"publisher","first-page":"1026","DOI":"10.1016\/j.autrev.2014.08.029","volume":"13","author":"C Ferri","year":"2014","unstructured":"Ferri C, Sebastiani M, Lo Monaco A et al (2014) Systemic sclerosis evolution of disease pathomorphosis and survival. Our experience on Italian patients\u2019 population and review of the literature. Autoimmun Rev 13:1026\u20131034. https:\/\/doi.org\/10.1016\/j.autrev.2014.08.029","journal-title":"Autoimmun Rev"},{"key":"8929_CR87","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1007\/s00296-016-3474-7","volume":"36","author":"P Elfving","year":"2016","unstructured":"Elfving P, Marjoniemi O, Niinisalo H et al (2016) Estimating the incidence of connective tissue diseases and vasculitides in a defined population in Northern Savo area in 2010. Rheumatol Int 36:917\u2013924. https:\/\/doi.org\/10.1007\/s00296-016-3474-7","journal-title":"Rheumatol Int"},{"key":"8929_CR88","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1007\/s12026-009-8153-2","volume":"47","author":"WW Eaton","year":"2010","unstructured":"Eaton WW, Pedersen MG, Atlad\u00f3ttir H\u00d3 et al (2010) The prevalence of 30 ICD-10 autoimmune diseases in Denmark. Immunol Res 47:228\u2013231. https:\/\/doi.org\/10.1007\/s12026-009-8153-2","journal-title":"Immunol Res"},{"key":"8929_CR89","doi-asserted-by":"publisher","first-page":"1362","DOI":"10.1002\/art.1780390814","volume":"39","author":"FC Arnett","year":"1996","unstructured":"Arnett FC, Howard RF, Tan F et al (1996) Increased prevalence of systemic sclerosis in a native american tribe in oklahoma. Association with an amerindian HLA haplotype. Arthritis Rheum 39:1362\u20131370. https:\/\/doi.org\/10.1002\/art.1780390814","journal-title":"Arthritis Rheum"},{"key":"8929_CR90","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1002\/acr.20656","volume":"64","author":"C Barnabe","year":"2012","unstructured":"Barnabe C, Joseph L, Belisle P et al (2012) Prevalence of systemic lupus erythematosus and systemic sclerosis in the First Nations population of Alberta, Canada. Arthritis Care Res 64:138\u2013143. https:\/\/doi.org\/10.1002\/acr.20656","journal-title":"Arthritis Care Res"},{"key":"8929_CR91","doi-asserted-by":"publisher","first-page":"734","DOI":"10.1002\/art.1780400421","volume":"40","author":"TJ Laing","year":"1997","unstructured":"Laing TJ, Gillespie BW, Toth MB et al (1997) Racial differences in scleroderma among women in Michigan. Arthritis Rheum 40:734\u2013742. https:\/\/doi.org\/10.1002\/art.1780400421","journal-title":"Arthritis Rheum"},{"key":"8929_CR92","doi-asserted-by":"publisher","first-page":"2246","DOI":"10.1002\/art.11073","volume":"48","author":"MD Mayes","year":"2003","unstructured":"Mayes MD, Lacey JV, Beebe-Dimmer J et al (2003) Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 48:2246\u20132255. https:\/\/doi.org\/10.1002\/art.11073","journal-title":"Arthritis Rheum"},{"key":"8929_CR93","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1073\/pnas.1906593116","volume":"117","author":"P Gourh","year":"2020","unstructured":"Gourh P, Safran SA, Alexander T et al (2020) HLA and autoantibodies define scleroderma subtypes and risk in African and European Americans and suggest a role for molecular mimicry. Proc Natl Acad Sci U S A 117:552\u2013562. https:\/\/doi.org\/10.1073\/pnas.1906593116","journal-title":"Proc Natl Acad Sci U S A"},{"key":"8929_CR94","doi-asserted-by":"publisher","first-page":"1684","DOI":"10.1093\/rheumatology\/kez486","volume":"59","author":"VK Jaeger","year":"2020","unstructured":"Jaeger VK, Tikly M, Xu D et al (2020) Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study. Rheumatology 59:1684\u20131694. https:\/\/doi.org\/10.1093\/rheumatology\/kez486","journal-title":"Rheumatology"},{"key":"8929_CR95","doi-asserted-by":"publisher","first-page":"1154","DOI":"10.1002\/acr.23861","volume":"71","author":"DF Moore","year":"2019","unstructured":"Moore DF, Kramer E, Eltaraboulsi R, Steen VD (2019) Increased morbidity and mortality of scleroderma in African Americans compared to non\u2013African Americans. Arthritis Care Res 71:1154\u20131163. https:\/\/doi.org\/10.1002\/acr.23861","journal-title":"Arthritis Care Res"},{"key":"8929_CR96","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1002\/art.38219","volume":"66","author":"V Sobanski","year":"2014","unstructured":"Sobanski V, Dauchet L, Lef\u00e8vre G et al (2014) Prevalence of anti\u2013RNA polymerase III antibodies in systemic sclerosis: new data from a French cohort and a systematic review and meta-analysis. Arthritis Rheumatol 66:407\u2013417. https:\/\/doi.org\/10.1002\/art.38219","journal-title":"Arthritis & Rheumatology"},{"key":"8929_CR97","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1007\/s10067-016-3533-1","volume":"36","author":"M Rubio-Rivas","year":"2017","unstructured":"Rubio-Rivas M, Moreno R, Corbella X (2017) Occupational and environmental scleroderma. Systematic review and meta-analysis Clin Rheumatol 36:569\u2013582. https:\/\/doi.org\/10.1007\/s10067-016-3533-1","journal-title":"Systematic review and meta-analysis Clin Rheumatol"},{"key":"8929_CR98","doi-asserted-by":"publisher","first-page":"62","DOI":"10.1016\/j.autrev.2018.11.001","volume":"18","author":"I Marie","year":"2019","unstructured":"Marie I (2019) Systemic sclerosis and exposure to heavy metals. Autoimmun Rev 18:62\u201372. https:\/\/doi.org\/10.1016\/j.autrev.2018.11.001","journal-title":"Autoimmun Rev"},{"key":"8929_CR99","doi-asserted-by":"publisher","first-page":"763","DOI":"10.1007\/s00420-009-0505-7","volume":"83","author":"ZD McCormic","year":"2010","unstructured":"McCormic ZD, Khuder SS, Aryal BK et al (2010) Occupational silica exposure as a risk factor for scleroderma: a meta-analysis. Int Arch Occup Environ Health 83:763\u2013769. https:\/\/doi.org\/10.1007\/s00420-009-0505-7","journal-title":"Int Arch Occup Environ Health"},{"key":"8929_CR100","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1016\/j.semarthrit.2018.06.009","volume":"48","author":"C Ferri","year":"2018","unstructured":"Ferri C, Artoni E, Sighinolfi GL et al (2018) High serum levels of silica nanoparticles in systemic sclerosis patients with occupational exposure: possible pathogenetic role in disease phenotypes. Semin Arthritis Rheum 48:475\u2013481. https:\/\/doi.org\/10.1016\/j.semarthrit.2018.06.009","journal-title":"Semin Arthritis Rheum"},{"key":"8929_CR101","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1097\/SLA.0000000000002990","volume":"269","author":"CJ Coroneos","year":"2019","unstructured":"Coroneos CJ, Selber JC, Offodile ACI et al (2019) US FDA breast implant postapproval studies: long-term outcomes in 99,993 patients. Ann Surg 269:30\u201336. https:\/\/doi.org\/10.1097\/SLA.0000000000002990","journal-title":"Ann Surg"},{"key":"8929_CR102","doi-asserted-by":"publisher","first-page":"808","DOI":"10.1111\/1346-8138.13292","volume":"43","author":"R Saigusa","year":"2016","unstructured":"Saigusa R, Asano Y, Nakamura K et al (2016) Association of anti-RNA polymerase III antibody and silicone breast implants in patients with systemic sclerosis. J Dermatol 43:808\u2013810. https:\/\/doi.org\/10.1111\/1346-8138.13292","journal-title":"J Dermatol"},{"key":"8929_CR103","doi-asserted-by":"crossref","unstructured":"Lazzaroni MG, Campochiaro C, Bertoldo E et al (2020) Association of anti-RNA polymerase III antibody with silicone breast implants rupture in a multicentre series of Italian patients with systemic sclerosis. Clin Exp Rheumatol","DOI":"10.1136\/annrheumdis-2019-eular.5726"},{"key":"8929_CR104","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1126\/science.1246886","volume":"343","author":"CG Joseph","year":"2014","unstructured":"Joseph CG, Darrah E, Shah AA et al (2014) Association of the autoimmune disease scleroderma with an immunologic response to cancer. Science 343:152\u2013157. https:\/\/doi.org\/10.1126\/science.1246886","journal-title":"Science"},{"key":"8929_CR105","doi-asserted-by":"publisher","first-page":"R53","DOI":"10.1186\/ar4486","volume":"16","author":"P Moinzadeh","year":"2014","unstructured":"Moinzadeh P, Fonseca C, Hellmich M et al (2014) Association of anti-RNA polymerase III autoantibodies and cancer in scleroderma. Arthritis Res Ther 16:R53. https:\/\/doi.org\/10.1186\/ar4486","journal-title":"Arthritis Res Ther"},{"key":"8929_CR106","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2020.102494","volume":"19","author":"M Hughes","year":"2020","unstructured":"Hughes M, Pauling JD, Armstrong-James L et al (2020) Gender-related differences in systemic sclerosis. Autoimmun Rev 19:102494. https:\/\/doi.org\/10.1016\/j.autrev.2020.102494","journal-title":"Autoimmun Rev"},{"key":"8929_CR107","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1136\/annrheumdis-2014-206386","volume":"75","author":"M Elhai","year":"2016","unstructured":"Elhai M, Avouac J, Walker UA et al (2016) A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study. Ann Rheum Dis 75:163\u2013169. https:\/\/doi.org\/10.1136\/annrheumdis-2014-206386","journal-title":"Ann Rheum Dis"},{"key":"8929_CR108","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1111\/j.1749-6632.2002.tb04221.x","volume":"966","author":"R Scorza","year":"2002","unstructured":"Scorza R, Caronni M, Bazzi S et al (2002) Post-menopause is the main risk factor for developing isolated pulmonary hypertension in systemic sclerosis. Ann N Y Acad Sci 966:238\u2013246. https:\/\/doi.org\/10.1111\/j.1749-6632.2002.tb04221.x","journal-title":"Ann N Y Acad Sci"},{"key":"8929_CR109","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1016\/j.semarthrit.2019.07.007","volume":"50","author":"J Ciaffi","year":"2020","unstructured":"Ciaffi J, van Leeuwen NM, Schoones JW et al (2020) Sex hormones and sex hormone-targeting therapies in systemic sclerosis: a systematic literature review. Semin Arthritis Rheum 50:140\u2013148. https:\/\/doi.org\/10.1016\/j.semarthrit.2019.07.007","journal-title":"Semin Arthritis Rheum"},{"key":"8929_CR110","doi-asserted-by":"publisher","first-page":"593","DOI":"10.1016\/j.jid.2019.07.719","volume":"140","author":"J Avouac","year":"2020","unstructured":"Avouac J, Pezet S, Gonzalez V et al (2020) Estrogens counteract the profibrotic effects of TGF-\u03b2 and their inhibition exacerbates experimental dermal fibrosis. J Invest Dermatol 140:593-601.e7. https:\/\/doi.org\/10.1016\/j.jid.2019.07.719","journal-title":"J Invest Dermatol"},{"key":"8929_CR111","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1186\/s13075-019-1870-6","volume":"21","author":"D Baker Frost","year":"2019","unstructured":"Baker Frost D, Wolf B, Peoples C et al (2019) Estradiol levels are elevated in older men with diffuse cutaneous SSc and are associated with decreased survival. Arthritis Res Ther 21:85. https:\/\/doi.org\/10.1186\/s13075-019-1870-6","journal-title":"Arthritis Res Ther"},{"key":"8929_CR112","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1080\/03009742.2018.1459830","volume":"48","author":"PE Carreira","year":"2019","unstructured":"Carreira PE, Carmona L, Joven BE et al (2019) Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database. Scand J Rheumatol 48:42\u201351. https:\/\/doi.org\/10.1080\/03009742.2018.1459830","journal-title":"Scand J Rheumatol"},{"key":"8929_CR113","doi-asserted-by":"publisher","first-page":"3380","DOI":"10.1093\/rheumatology\/keaa127","volume":"59","author":"P Moinzadeh","year":"2020","unstructured":"Moinzadeh P, Kuhr K, Siegert E et al (2020) Older age onset of systemic sclerosis\u2013accelerated disease progression in all disease subsets. Rheumatology 59:3380\u20133389. https:\/\/doi.org\/10.1093\/rheumatology\/keaa127","journal-title":"Rheumatology"},{"key":"8929_CR114","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1007\/s00393-018-0492-8","volume":"78","author":"YH Lee","year":"2019","unstructured":"Lee YH (2019) Overall and sex- and disease subtype-specific mortality in patients with systemic sclerosis. Z Rheumatol 78:195\u2013201. https:\/\/doi.org\/10.1007\/s00393-018-0492-8","journal-title":"Z Rheumatol"},{"key":"8929_CR115","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 (2007) Changes in causes of death in systemic sclerosis, 1972\u20132002. Ann Rheum Dis 66:940\u2013944. https:\/\/doi.org\/10.1136\/ard.2006.066068","journal-title":"Ann Rheum Dis"},{"key":"8929_CR116","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 et al (2017) Mapping and predicting mortality from systemic sclerosis. Ann Rheum Dis 76:1897\u20131905. https:\/\/doi.org\/10.1136\/annrheumdis-2017-211448","journal-title":"Ann Rheum Dis"},{"key":"8929_CR117","doi-asserted-by":"publisher","first-page":"1809","DOI":"10.1136\/ard.2009.114264","volume":"69","author":"AJ Tyndall","year":"2010","unstructured":"Tyndall AJ, Bannert B, Vonk M et al (2010) Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis 69:1809\u20131815. https:\/\/doi.org\/10.1136\/ard.2009.114264","journal-title":"Ann Rheum Dis"},{"key":"8929_CR118","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1186\/s13075-019-1867-1","volume":"21","author":"MR Pokeerbux","year":"2019","unstructured":"Pokeerbux MR, Giovannelli J, Dauchet L et al (2019) Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature. Arthritis Res Ther 21:86. https:\/\/doi.org\/10.1186\/s13075-019-1867-1","journal-title":"Arthritis Res Ther"},{"key":"8929_CR119","doi-asserted-by":"publisher","first-page":"648","DOI":"10.1136\/annrheumdis-2018-213455","volume":"78","author":"W Wu","year":"2019","unstructured":"Wu W, Jordan S, Graf N et al (2019) Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort. Ann Rheum Dis 78:648\u2013656. https:\/\/doi.org\/10.1136\/annrheumdis-2018-213455","journal-title":"Ann Rheum Dis"},{"key":"8929_CR120","doi-asserted-by":"publisher","first-page":"2660","DOI":"10.1002\/1529-0131(199912)42:12<2660::AID-ANR23>3.0.CO;2-N","volume":"42","author":"C Bryan","year":"1999","unstructured":"Bryan C, Knight C, Black CM, Silman AJ (1999) Prediction of five-year survival following presentation with scleroderma: development of a simple model using three disease factors at first visit. Arthritis Rheum 42:2660\u20132665. https:\/\/doi.org\/10.1002\/1529-0131(199912)42:12%3c2660::AID-ANR23%3e3.0.CO;2-N","journal-title":"Arthritis Rheum"},{"key":"8929_CR121","doi-asserted-by":"publisher","first-page":"1788","DOI":"10.1136\/ard.2010.144360","volume":"70","author":"J Fransen","year":"2011","unstructured":"Fransen J, Popa-Diaconu D, Hesselstrand R et al (2011) Clinical prediction of 5-year survival in systemic sclerosis: validation of a simple prognostic model in EUSTAR centres. Ann Rheum Dis 70:1788\u20131792. https:\/\/doi.org\/10.1136\/ard.2010.144360","journal-title":"Ann Rheum Dis"},{"key":"8929_CR122","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2019.101469","volume":"34","author":"A Quinlivan","year":"2020","unstructured":"Quinlivan A, Ross L, Proudman S (2020) Systemic sclerosis: advances towards stratified medicine. Best Pract Res Clin Rheumatol 34:101469. https:\/\/doi.org\/10.1016\/j.berh.2019.101469","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"8929_CR123","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2019.101428","volume":"33","author":"S Bellando-Randone","year":"2019","unstructured":"Bellando-Randone S, Matucci-Cerinic M (2019) Very early systemic sclerosis. Best Pract Res Clin Rheumatol 33:101428. https:\/\/doi.org\/10.1016\/j.berh.2019.101428","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"8929_CR124","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1093\/rheumatology\/keu288","volume":"54","author":"D Khanna","year":"2015","unstructured":"Khanna D, Furst DE, Allanore Y et al (2015) Twenty-two points to consider for clinical trials in systemic sclerosis, based on EULAR standards. Rheumatology 54:144\u2013151. https:\/\/doi.org\/10.1093\/rheumatology\/keu288","journal-title":"Rheumatology"},{"key":"8929_CR125","first-page":"1194","volume":"34","author":"D Furst","year":"2007","unstructured":"Furst D, Khanna D, Matucci-Cerinic M et al (2007) Systemic sclerosis-continuing progress in developing clinical measures of response. J Rheumatol 34:1194\u20131200","journal-title":"J Rheumatol"},{"key":"8929_CR126","doi-asserted-by":"publisher","first-page":"703","DOI":"10.1136\/ard.2007.078923","volume":"67","author":"D Khanna","year":"2008","unstructured":"Khanna D, Lovell DJ, Giannini E et al (2008) Development of a provisional core set of response measures for clinical trials of systemic sclerosis. Ann Rheum Dis 67:703\u2013709. https:\/\/doi.org\/10.1136\/ard.2007.078923","journal-title":"Ann Rheum Dis"},{"key":"8929_CR127","doi-asserted-by":"publisher","first-page":"1449","DOI":"10.1002\/acr.24027","volume":"72","author":"D Sumpton","year":"2020","unstructured":"Sumpton D, Bigot A, Sautenet B et al (2020) Scope and consistency of outcomes reported in trials of patients with systemic sclerosis. Arthritis Care Res 72:1449\u20131458. https:\/\/doi.org\/10.1002\/acr.24027","journal-title":"Arthritis Care Res"},{"key":"8929_CR128","doi-asserted-by":"publisher","first-page":"2356","DOI":"10.3899\/jrheum.090372","volume":"36","author":"D Khanna","year":"2009","unstructured":"Khanna D, Distler O, Avouac J et al (2009) Measures of response in clinical trials of systemic sclerosis: the Combined Response Index for Systemic Sclerosis (CRISS) and Outcome Measures in Pulmonary Arterial Hypertension Related to Systemic Sclerosis (EPOSS). J Rheumatol 36:2356\u20132361. https:\/\/doi.org\/10.3899\/jrheum.090372","journal-title":"J Rheumatol"},{"key":"8929_CR129","doi-asserted-by":"publisher","first-page":"167","DOI":"10.1002\/acr.22804","volume":"68","author":"D Khanna","year":"2016","unstructured":"Khanna D, Berrocal VJ, Giannini EH et al (2016) The American college of rheumatology provisional composite response index for clinical trials in early diffuse cutaneous systemic sclerosis. Arthritis Care Res 68:167\u2013178. https:\/\/doi.org\/10.1002\/acr.22804","journal-title":"Arthritis Care Res"},{"key":"8929_CR130","doi-asserted-by":"publisher","unstructured":"Zheng B, Wang M, McKenna K et al (2022) Agreement between physician evaluation and the Composite Response Index in Diffuse Cutaneous Systemic Sclerosis (CRISS). Arthritis Care Res n\/a. https:\/\/doi.org\/10.1002\/acr.24638","DOI":"10.1002\/acr.24638"},{"key":"8929_CR131","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1186\/s13075-020-02220-0","volume":"22","author":"B Zheng","year":"2020","unstructured":"Zheng B, Hudson M, Wang M et al (2020) Immunosuppressive treatment in diffuse cutaneous systemic sclerosis is associated with an improved composite response index (CRISS). Arthritis Res Ther 22:132. https:\/\/doi.org\/10.1186\/s13075-020-02220-0","journal-title":"Arthritis Res Ther"},{"key":"8929_CR132","doi-asserted-by":"publisher","unstructured":"Khanna D, Huang S, Lin CJF, Spino C (2020) New composite endpoint in early diffuse cutaneous systemic sclerosis: revisiting the provisional American College of Rheumatology Composite Response Index in Systemic Sclerosis. Ann Rheum Dis. https:\/\/doi.org\/10.1136\/annrheumdis-2020-219100","DOI":"10.1136\/annrheumdis-2020-219100"},{"key":"8929_CR133","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1703327","author":"KM Sullivan","year":"2018","unstructured":"Sullivan KM, Goldmuntz EA, Keyes-Elstein L et al (2018) Myeloablative autologous stem-cell transplantation for severe scleroderma. N Engl J Med. https:\/\/doi.org\/10.1056\/NEJMoa1703327","journal-title":"N Engl J Med"},{"key":"8929_CR134","doi-asserted-by":"publisher","first-page":"937","DOI":"10.1016\/j.bbmt.2018.01.020","volume":"24","author":"R Shouval","year":"2018","unstructured":"Shouval R, Furie N, Raanani P et al (2018) Autologous hematopoietic stem cell transplantation for systemic sclerosis: a systematic review and meta-analysis. Biol Blood Marrow Transplant 24:937\u2013944. https:\/\/doi.org\/10.1016\/j.bbmt.2018.01.020","journal-title":"Biol Blood Marrow Transplant"},{"key":"8929_CR135","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1038\/s41409-021-01355-1","volume":"1","author":"N Ait Abdallah","year":"2021","unstructured":"Ait Abdallah N, Wang M, Lansiaux P et al (2021) Long term outcomes of the French ASTIS systemic sclerosis cohort using the global rank composite score. Bone Marrow Transplant 1\u20139. https:\/\/doi.org\/10.1038\/s41409-021-01355-1","journal-title":"Bone Marrow Transplant"},{"key":"8929_CR136","doi-asserted-by":"publisher","first-page":"e173","DOI":"10.1016\/S2665-9913(20)30007-2","volume":"2","author":"F Del Galdo","year":"2020","unstructured":"Del Galdo F, Hartley C, Allanore Y (2020) Randomised controlled trials in systemic sclerosis: patient selection and endpoints for next generation trials. Lancet Rheumatol 2:e173\u2013e184. https:\/\/doi.org\/10.1016\/S2665-9913(20)30007-2","journal-title":"The Lancet Rheumatology"},{"key":"8929_CR137","doi-asserted-by":"publisher","first-page":"1049","DOI":"10.1002\/art.41246","volume":"72","author":"V Nagaraja","year":"2020","unstructured":"Nagaraja V, Matucci-Cerinic M, Furst DE et al (2020) Current and future outlook on disease modification and defining low disease activity in systemic sclerosis. Arthritis Rheumatol 72:1049\u20131058. https:\/\/doi.org\/10.1002\/art.41246","journal-title":"Arthritis & Rheumatology"},{"key":"8929_CR138","doi-asserted-by":"publisher","first-page":"592","DOI":"10.1136\/ard.60.6.592","volume":"60","author":"G Valentini","year":"2001","unstructured":"Valentini G, Rossa AD, Bombardieri S et al (2001) European multicentre study to define disease activity criteria for systemic sclerosis. II. Identification of disease activity variables and development of preliminary activity indexes. Ann Rheum Dis 60:592\u2013598. https:\/\/doi.org\/10.1136\/ard.60.6.592","journal-title":"Ann Rheum Dis"},{"key":"8929_CR139","doi-asserted-by":"publisher","first-page":"270","DOI":"10.1136\/annrheumdis-2016-209768","volume":"76","author":"G Valentini","year":"2017","unstructured":"Valentini G, Iudici M, Walker UA et al (2017) The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index. Ann Rheum Dis 76:270\u2013276. https:\/\/doi.org\/10.1136\/annrheumdis-2016-209768","journal-title":"Ann Rheum Dis"},{"issue":"Suppl 125","key":"8929_CR140","first-page":"53","volume":"38","author":"M Doyen","year":"2020","unstructured":"Doyen M, Houssiau FA, Lauwerys BR, Vanthuyne M (2020) Comparison of the disease activity score and the revised EUSTAR activity index in diffuse cutaneous systemic sclerosis patients. Clin Exp Rheumatol 38(Suppl 125):53\u201358","journal-title":"Clin Exp Rheumatol"},{"key":"8929_CR141","doi-asserted-by":"publisher","first-page":"807","DOI":"10.1136\/annrheumdis-2018-214764","volume":"78","author":"N Ferdowsi","year":"2019","unstructured":"Ferdowsi N, Huq M, Stevens W et al (2019) Development and validation of the Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI): a novel instrument to quantify organ damage in systemic sclerosis. Ann Rheum Dis 78:807. https:\/\/doi.org\/10.1136\/annrheumdis-2018-214764","journal-title":"Ann Rheum Dis"},{"key":"8929_CR142","doi-asserted-by":"publisher","first-page":"e98","DOI":"10.1136\/annrheumdis-2019-215588","volume":"79","author":"S Jain","year":"2020","unstructured":"Jain S, Sharma SK (2020) Differentiating disease activity from damage in systemic sclerosis: it\u2019s still early days! Ann Rheum Dis 79:e98\u2013e98. https:\/\/doi.org\/10.1136\/annrheumdis-2019-215588","journal-title":"Ann Rheum Dis"},{"key":"8929_CR143","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1136\/annrheumdis-2021-eular.582","volume":"80","author":"MG Lazzaroni","year":"2021","unstructured":"Lazzaroni MG, Breda M, Franceschini F, Air\u00f2 P (2021) Op0273 the scleroderma clinical trials consortium damage index (sctc-Di) in a systemic sclerosis cohort with 10-years follow-up. Ann Rheum Dis 80:166\u2013166. https:\/\/doi.org\/10.1136\/annrheumdis-2021-eular.582","journal-title":"Ann Rheum Dis"},{"key":"8929_CR144","first-page":"S42","volume":"21","author":"TA Medsger","year":"2003","unstructured":"Medsger TA, Bombardieri S, Czirjak L et al (2003) Assessment of disease severity and prognosis. Clin Exp Rheumatol 21:S42-46","journal-title":"Clin Exp Rheumatol"},{"key":"8929_CR145","doi-asserted-by":"publisher","first-page":"726","DOI":"10.1002\/art.38277","volume":"66","author":"D Rimar","year":"2014","unstructured":"Rimar D, Rosner I, Nov Y et al (2014) Brief report: lysyl oxidase is a potential biomarker of fibrosis in systemic sclerosis. Arthritis Rheumatol 66:726\u2013730. https:\/\/doi.org\/10.1002\/art.38277","journal-title":"Arthritis Rheumatol"},{"key":"8929_CR146","doi-asserted-by":"publisher","unstructured":"Gheita TA, Hussein H (2012) Cartilage Oligomeric Matrix Protein (COMP) in systemic sclerosis (SSc): role in disease severity and subclinical rheumatoid arthritis overlap. Joint Bone Spine 79:51\u20136. https:\/\/doi.org\/10.1016\/j.jbspin.2011.02.022","DOI":"10.1016\/j.jbspin.2011.02.022"},{"key":"8929_CR147","doi-asserted-by":"publisher","first-page":"1620","DOI":"10.1002\/acr.20591","volume":"63","author":"M Hinchcliff","year":"2011","unstructured":"Hinchcliff M, Beaumont JL, Thavarajah K et al (2011) Validity of two new patient-reported outcome measures in systemic sclerosis: patient-reported outcomes measurement information system 29-item health profile and functional assessment of chronic illness therapy-dyspnea short form. Arthritis Care Res (Hoboken) 63:1620\u20131628. https:\/\/doi.org\/10.1002\/acr.20591","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"8929_CR148","doi-asserted-by":"publisher","first-page":"1117","DOI":"10.1136\/ard.60.12.1117","volume":"60","author":"AJ Geirsson","year":"2001","unstructured":"Geirsson AJ, Wollheim FA, Akesson A (2001) Disease severity of 100 patients with systemic sclerosis over a period of 14 years: using a modified Medsger scale. Ann Rheum Dis 60:1117\u20131122. https:\/\/doi.org\/10.1136\/ard.60.12.1117","journal-title":"Ann Rheum Dis"},{"key":"8929_CR149","doi-asserted-by":"publisher","first-page":"1510","DOI":"10.3899\/jrheum.151440","volume":"43","author":"D Harel","year":"2016","unstructured":"Harel D, Hudson M, Iliescu A et al (2016) Summed and weighted summary scores for the Medsger disease severity scale compared with the physician\u2019s global assessment of disease severity in systemic sclerosis. J Rheumatol 43:1510\u20131518. https:\/\/doi.org\/10.3899\/jrheum.151440","journal-title":"J Rheumatol"},{"key":"8929_CR150","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1177\/2397198319886496","volume":"5","author":"JD Pauling","year":"2020","unstructured":"Pauling JD, Caetano J, Campochiaro C et al (2020) Patient-reported outcome instruments in clinical trials of systemic sclerosis. J Scleroderma Relat Disord 5:90\u2013102. https:\/\/doi.org\/10.1177\/2397198319886496","journal-title":"Journal of Scleroderma and Related Disorders"},{"key":"8929_CR151","doi-asserted-by":"publisher","first-page":"1459","DOI":"10.1002\/acr.24053","volume":"72","author":"L Ross","year":"2020","unstructured":"Ross L, Stevens W, Wilson M et al (2020) Can patient-reported symptoms be used to measure disease activity in systemic sclerosis? Arthritis Care Res (Hoboken) 72:1459\u20131465. https:\/\/doi.org\/10.1002\/acr.24053","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"8929_CR152","doi-asserted-by":"publisher","first-page":"S98","DOI":"10.1002\/acr.20598","volume":"63","author":"J Pope","year":"2011","unstructured":"Pope J (2011) Measures of systemic sclerosis (scleroderma): Health Assessment Questionnaire (HAQ) and Scleroderma HAQ (SHAQ), Physician- and Patient-Rated Global Assessments, Symptom Burden Index (SBI), University of California, Los Angeles, Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0, Baseline Dyspnea Index (BDI) and Transition Dyspnea Index (TDI) (Mahler\u2019s Index), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), and Raynaud\u2019s Condition Score (RCS). Arthritis Care Res 63:S98\u2013S111. https:\/\/doi.org\/10.1002\/acr.20598","journal-title":"Arthritis Care Res"},{"key":"8929_CR153","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1186\/s13075-020-02329-2","volume":"22","author":"Y Allanore","year":"2020","unstructured":"Allanore Y, Bozzi S, Terlinden A et al (2020) Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database. Arthritis Res Ther 22:257. https:\/\/doi.org\/10.1186\/s13075-020-02329-2","journal-title":"Arthritis Res Ther"},{"key":"8929_CR154","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1093\/rheumatology\/kex182","volume":"57","author":"VK Jaeger","year":"2018","unstructured":"Jaeger VK, Distler O, Maurer B et al (2018) Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group. Rheumatology 57:441\u2013450. https:\/\/doi.org\/10.1093\/rheumatology\/kex182","journal-title":"Rheumatology"},{"key":"8929_CR155","doi-asserted-by":"publisher","first-page":"1257","DOI":"10.1002\/art.24730","volume":"61","author":"D Khanna","year":"2009","unstructured":"Khanna D, Hays RD, Maranian P et al (2009) Reliability and validity of the University of California, Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract Instrument. Arthritis Rheum 61:1257\u20131263. https:\/\/doi.org\/10.1002\/art.24730","journal-title":"Arthritis Rheum"},{"key":"8929_CR156","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.20688","author":"J Sibeoni","year":"2021","unstructured":"Sibeoni J, Dunogu\u00e9 B, Dupont A et al (2021) Development and validation of a Patient-Reported Outcome in systemic sclerosis: the Hand scleroDerma lived Experience Scale (HAnDE Scale). Br J Dermatol. https:\/\/doi.org\/10.1111\/bjd.20688","journal-title":"Br J Dermatol"},{"key":"8929_CR157","doi-asserted-by":"publisher","unstructured":"Jones PW, Quirk FH, Baveystock CM (1991) The St George\u2019s respiratory questionnaire. Respir Med 85(Suppl_B):25\u201331; discussion 33\u201337. https:\/\/doi.org\/10.1016\/s0954-6111(06)80166-6","DOI":"10.1016\/s0954-6111(06)80166-6"},{"key":"8929_CR158","doi-asserted-by":"publisher","first-page":"64","DOI":"10.3899\/jrheum.140143","volume":"42","author":"ME Hinchcliff","year":"2015","unstructured":"Hinchcliff ME, Beaumont JL, Carns MA et al (2015) Longitudinal evaluation of PROMIS-29 and FACIT-Dyspnea short forms in systemic sclerosis. J Rheumatol 42:64\u201372. https:\/\/doi.org\/10.3899\/jrheum.140143","journal-title":"J Rheumatol"},{"key":"8929_CR159","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1903076","author":"O Distler","year":"2019","unstructured":"Distler O, Highland KB, Gahlemann M et al (2019) Nintedanib for systemic sclerosis\u2013associated interstitial lung disease. N Engl J Med. https:\/\/doi.org\/10.1056\/NEJMoa1903076","journal-title":"N Engl J Med"}],"container-title":["Clinical Reviews in Allergy &amp; Immunology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12016-022-08929-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s12016-022-08929-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s12016-022-08929-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,10]],"date-time":"2023-05-10T08:15:53Z","timestamp":1683706553000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s12016-022-08929-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,3,7]]},"references-count":159,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,6]]}},"alternative-id":["8929"],"URL":"https:\/\/doi.org\/10.1007\/s12016-022-08929-x","relation":{},"ISSN":["1559-0267"],"issn-type":[{"value":"1559-0267","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,3,7]]},"assertion":[{"value":"31 January 2022","order":1,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 March 2022","order":2,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 July 2022","order":3,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Update","order":4,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The original version of this paper was updated to present the missing Open Access funding note.","order":5,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Dr. Paolo Air\u00f2 reports receiving personal fees from Bristol Myers Squibb and from Boehringer Ingelheim, and non-financial support from CSL Behring, SOBI, Janssen, Roche, and Sanofi. Personal fees include speakers bureau, consultancy (<\u20092000 EURO); non-financial support includes travel expenses (<\u20091000 EURO). No other potential conflict of interest relevant to this article was reported.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}}]}}