{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T18:32:43Z","timestamp":1792089163390,"version":"4.4.0"},"reference-count":107,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T00:00:00Z","timestamp":1599177600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001826","name":"ZonMw","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001826","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"DOI":"10.3389\/fimmu.2020.01990","type":"journal-article","created":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T13:52:15Z","timestamp":1599227535000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":23,"title":["Interstitial Lung Disease in Patients With Systemic Sclerosis: Toward Personalized-Medicine-Based Prediction and Drug Screening Models of Systemic Sclerosis-Related Interstitial Lung Disease (SSc-ILD)"],"prefix":"10.3389","volume":"11","author":[{"given":"Padmini","family":"Khedoe","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emiel","family":"Marges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pieter","family":"Hiemstra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maarten","family":"Ninaber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miranda","family":"Geelhoed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2020,9,4]]},"reference":[{"key":"B1","first-page":"198","article-title":"Systemic sclerosis: an update.","volume":"66","author":"Varga","year":"2008","journal-title":"Bull NYU Hosp Jt Dis."},{"key":"B2","doi-asserted-by":"publisher","first-page":"940","DOI":"10.1136\/ard.2006.066068","article-title":"Changes in causes of death in systemic sclerosis, 1972-2002.","volume":"66","author":"Steen","year":"2007","journal-title":"Ann Rheum Dis."},{"key":"B3","doi-asserted-by":"crossref","first-page":"1809","DOI":"10.1136\/ard.2009.114264","article-title":"Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.","volume":"69","author":"Tyndall","year":"2010","journal-title":"Ann Rheum Dis."},{"key":"B4","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1136\/annrheumdis-2016-209909","article-title":"Update of EULAR recommendations for the treatment of systemic sclerosis.","volume":"76","author":"Kowal-Bielecka","year":"2017","journal-title":"Ann Rheum Dis."},{"key":"B5","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.addr.2017.12.013","article-title":"Engineered cell and tissue models of pulmonary fibrosis.","volume":"129","author":"Sundarakrishnan","year":"2018","journal-title":"Adv Drug Deliv Rev."},{"key":"B6","doi-asserted-by":"publisher","first-page":"1941","DOI":"10.1016\/S0140-6736(17)30866-8","article-title":"Idiopathic pulmonary fibrosis.","volume":"389","author":"Richeldi","year":"2017","journal-title":"Lancet."},{"key":"B7","doi-asserted-by":"publisher","first-page":"1811","DOI":"10.1056\/NEJMra1705751","article-title":"Idiopathic pulmonary fibrosis.","volume":"378","author":"Lederer","year":"2018","journal-title":"N Engl J Med."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1161","DOI":"10.1164\/rccm.201312-2221PP","article-title":"Revealing the pathogenic and aging-related mechanisms of the enigmatic idiopathic pulmonary fibrosis. an integral model.","volume":"189","author":"Selman","year":"2014","journal-title":"Am J Respir Crit Care Med."},{"key":"B9","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1016\/j.amjms.2019.01.010","article-title":"Epithelial injury and dysfunction in the pathogenesis of idiopathic pulmonaryfibrosis.","volume":"357","author":"Winters","year":"2019","journal-title":"Am J Med Sci."},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.3390\/medicina55040083","article-title":"Epithelial(-)mesenchymal transition in the pathogenesis of idiopathic pulmonary fibrosis.","volume":"55","author":"Salton","year":"2019","journal-title":"Medicina (Kaunas)."},{"key":"B11","doi-asserted-by":"publisher","first-page":"31","DOI":"10.29245\/2689-999X\/2019\/2.1149","article-title":"Epithelial-mesenchymal transition contributes to pulmonary fibrosis via aberrant epithelial\/fibroblastic cross-talk.","volume":"3","author":"Hill","year":"2019","journal-title":"J Lung Health Dis."},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.3390\/ijms19082460","article-title":"TGF-beta signaling in lung health and disease.","volume":"19","author":"Saito","year":"2018","journal-title":"Int J Mol Sci."},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1155\/2016\/6064830","article-title":"Reassessing the role of the active TGF-beta1 as a biomarker in systemic sclerosis: association of serum levels with clinical manifestations.","volume":"2016","author":"Dantas","year":"2016","journal-title":"Dis Markers."},{"key":"B14","doi-asserted-by":"publisher","first-page":"111","DOI":"10.1513\/pats.201203-023AW","article-title":"The impact of TGF-beta on lung fibrosis: from targeting to biomarkers.","volume":"9","author":"Fernandez","year":"2012","journal-title":"Proc Am Thorac Soc."},{"key":"B15","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1038\/s41573-019-0040-5","article-title":"Targeting metabolic dysregulation for fibrosis therapy.","volume":"19","author":"Zhao","year":"2020","journal-title":"Nat Rev Drug Discov."},{"key":"B16","doi-asserted-by":"publisher","first-page":"2211","DOI":"10.1016\/j.cca.2011.08.008","article-title":"Plasma CCN2 (connective tissue growth factor; CTGF) is a potential biomarker in idiopathic pulmonary fibrosis (IPF).","volume":"412","author":"Kono","year":"2011","journal-title":"Clinica Chimica Acta."},{"key":"B17","doi-asserted-by":"publisher","first-page":"S24","DOI":"10.1186\/1755-1536-5-S1-S24","article-title":"CTGF is a central mediator of tissue remodeling and fibrosis and its inhibition can reverse the process of fibrosis.","volume":"5","author":"Lipson","year":"2012","journal-title":"Fibrogenesis Tissue Repair."},{"key":"B18","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1164\/rccm.201603-0568OC","article-title":"Differential expression of VEGF-Axxx isoforms is critical for development of pulmonary fibrosis.","volume":"196","author":"Barratt","year":"2017","journal-title":"Am J Respir Crit Care Med."},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.3390\/ijms19051269","article-title":"VEGF (vascular endothelial growth factor) and fibrotic lung disease.","volume":"19","author":"Barratt","year":"2018","journal-title":"Int J Mol Sci."},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00876-2018","article-title":"Use of three-dimensional organoids and lung-on-a-chip methods to study lung development, regeneration and disease.","volume":"52","author":"Gkatzis","year":"2018","journal-title":"Eur Respir J."},{"key":"B21","article-title":"Airway organoids as models of human disease.","author":"Van Der Vaart","year":"2020","journal-title":"J Intern Med."},{"key":"B22","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1016\/j.stem.2014.07.012","article-title":"Repair and regeneration of the respiratory system: complexity, plasticity, and mechanisms of lung stem cell function.","volume":"15","author":"Hogan","year":"2014","journal-title":"Cell Stem Cell."},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.00323","article-title":"Using lung organoids to investigate epithelial barrier complexity and IL-17 signaling during respiratory infection.","volume":"10","author":"Paolicelli","year":"2019","journal-title":"Front Immunol."},{"key":"B24","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1016\/j.biomaterials.2016.10.046","article-title":"Human airway organoid engineering as a step toward lung regeneration and disease modeling.","volume":"113","author":"Tan","year":"2017","journal-title":"Biomaterials."},{"key":"B25","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1165\/rcmb.2018-0390OC","article-title":"Nascent lung organoids reveal epithelium- and bone morphogenetic protein-mediated suppression of fibroblast activation.","volume":"61","author":"Tan","year":"2019","journal-title":"Am J Respir Cell Mol Biol."},{"key":"B26","doi-asserted-by":"publisher","first-page":"L14","DOI":"10.1152\/ajplung.00400.2018","article-title":"TGF-beta activation impairs fibroblast ability to support adult lung epithelial progenitor cell organoid formation.","volume":"317","author":"Ng-Blichfeldt","year":"2019","journal-title":"Am J Physiol Lung Cell Mol Physiol."},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-014-0411-1","article-title":"Recent advances in understanding the pathogenesis of scleroderma-interstitial lung disease.","volume":"16","author":"Akter","year":"2014","journal-title":"Curr Rheumatol Rep."},{"key":"B28","doi-asserted-by":"publisher","first-page":"102","DOI":"10.1183\/09059180.00003214","article-title":"Cellular interactions in the pathogenesis of interstitial lung diseases.","volume":"24","author":"Bagnato","year":"2015","journal-title":"Eur Respir Rev."},{"key":"B29","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1016\/j.semarthrit.2008.10.009","article-title":"Bronchoalveolar lavage fluid in scleroderma interstitial lung disease: technical aspects and clinical correlations: review of the literature.","volume":"40","author":"Kowal-Bielecka","year":"2010","journal-title":"Semin Arthritis Rheum."},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1186\/1755-1536-4-15","article-title":"Altered monocyte and fibrocyte phenotype and function in scleroderma interstitial lung disease: reversal by caveolin-1 scaffolding domain peptide.","volume":"4","author":"Tourkina","year":"2011","journal-title":"Fibrogenesis Tissue Repair."},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms11703","article-title":"Tenascin-C drives persistence of organ fibrosis.","volume":"7","author":"Bhattacharyya","year":"2016","journal-title":"Nat Commun."},{"key":"B32","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0102974","article-title":"Regulation of the innate immune response by fibronectin: synergism between the III-1 and EDA domains.","volume":"9","author":"Kelsh","year":"2014","journal-title":"PLoS One."},{"key":"B33","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1016\/j.imlet.2017.09.011","article-title":"Endogenous ligands of TLR4 promote unresolving tissue fibrosis: Implications for systemic sclerosis and its targeted therapy.","volume":"195","author":"Bhattacharyya","year":"2018","journal-title":"Immunol Lett."},{"key":"B34","doi-asserted-by":"publisher","DOI":"10.3390\/ijms18122527","article-title":"Role of MicroRNAs in TGF-beta signaling pathway-mediated pulmonary fibrosis.","volume":"18","author":"Kang","year":"2017","journal-title":"Int J Mol Sci."},{"key":"B35","doi-asserted-by":"publisher","first-page":"1188","DOI":"10.1136\/annrheumdis-2013-204522","article-title":"Effects and safety of rituximab in systemic sclerosis: an analysis from the European Scleroderma Trial and Research (EUSTAR) group.","volume":"74","author":"Jordan","year":"2015","journal-title":"Ann Rheum Dis."},{"key":"B36","doi-asserted-by":"publisher","first-page":"3167","DOI":"10.1002\/art.22847","article-title":"cell infiltration in systemic sclerosis-associated interstitial lung disease.","volume":"56","author":"Lafyatis","year":"2007","journal-title":"Arthritis Rheum."},{"key":"B37","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2015.00272","article-title":"Pathogenesis of systemic sclerosis.","volume":"6","author":"Pattanaik","year":"2015","journal-title":"Front Immunol."},{"key":"B38","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1038\/nrrheum.2014.149","article-title":"Interstitial lung disease in connective tissue disease\u2013mechanisms and management.","volume":"10","author":"Wells","year":"2014","journal-title":"Nat Rev Rheumatol."},{"key":"B39","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1111\/bjd.17886","article-title":"Anti-C1q autoantibodies are frequently detected in patients with systemic sclerosis associated with pulmonary fibrosis.","volume":"181","author":"Liaskos","year":"2019","journal-title":"Br J Dermatol."},{"key":"B40","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1164\/rccm.201809-1775OC","article-title":"The transferrin receptor CD71 delineates functionally distinct airway macrophage subsets during idiopathic pulmonary fibrosis.","volume":"200","author":"Allden","year":"2019","journal-title":"Am J Respir Crit Care Med."},{"key":"B41","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02441-2018","article-title":"Proliferating SPP1\/MERTK-expressing macrophages in idiopathic pulmonary fibrosis.","volume":"54","author":"Morse","year":"2019","journal-title":"Eur Respir J."},{"key":"B42","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3007096","article-title":"Chitinase 3-like 1 suppresses injury and promotes fibroproliferative responses in Mammalian lung fibrosis.","volume":"6","author":"Zhou","year":"2014","journal-title":"Sci Transl Med."},{"key":"B43","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1016\/j.clim.2010.06.017","article-title":"Alternatively activated alveolar macrophages in pulmonary fibrosis-mediator production and intracellular signal transduction.","volume":"137","author":"Pechkovsky","year":"2010","journal-title":"Clin Immunol."},{"key":"B44","doi-asserted-by":"crossref","first-page":"1517","DOI":"10.1164\/rccm.201712-2410OC","article-title":"Single-cell transcriptomic analysis of human lung provides insights into the pathobiology of pulmonary fibrosis.","volume":"199","author":"Reyfman","year":"2019","journal-title":"Am J Respir Crit Care Med."},{"key":"B45","doi-asserted-by":"publisher","first-page":"1053","DOI":"10.1002\/art.39569","article-title":"Editorial: extracellular matrix: the common thread of disease progression in fibrosis?","volume":"68","author":"Kolb","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"B46","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0033-2018","article-title":"Matrix abnormalities in pulmonary fibrosis.","volume":"27","author":"Upagupta","year":"2018","journal-title":"Eur Respir Rev."},{"key":"B47","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-015-0308-1","article-title":"Association of elevated alpha-defensin levels with interstitial pneumonia in patients with systemic sclerosis.","volume":"16","author":"Sakamoto","year":"2015","journal-title":"Respir Res."},{"key":"B48","doi-asserted-by":"publisher","first-page":"812","DOI":"10.1038\/labinvest.2010.73","article-title":"Circulating monocytes from systemic sclerosis patients with interstitial lung disease show an enhanced profibrotic phenotype.","volume":"90","author":"Mathai","year":"2010","journal-title":"Lab Invest."},{"key":"B49","doi-asserted-by":"publisher","first-page":"435","DOI":"10.3899\/jrheum.120725","article-title":"Serum interleukin 6 is predictive of early functional decline and mortality in interstitial lung disease associated with systemic sclerosis.","volume":"40","author":"De Lauretis","year":"2013","journal-title":"J Rheumatol."},{"key":"B50","doi-asserted-by":"publisher","first-page":"1171","DOI":"10.1183\/13993003.01826-2015","article-title":"Peripheral blood myeloid-derived suppressor cells reflect disease status in idiopathic pulmonary fibrosis.","volume":"48","author":"Fernandez","year":"2016","journal-title":"Eur Respir J."},{"key":"B51","doi-asserted-by":"publisher","DOI":"10.1002\/cti2.1131","article-title":"Comparison of two human organoid models of lung and intestinal inflammation reveals Toll-like receptor signalling activation and monocyte recruitment.","volume":"9","author":"Jose","year":"2020","journal-title":"Clin Transl Immunol."},{"key":"B52","doi-asserted-by":"publisher","first-page":"952","DOI":"10.1126\/science.aaw6985","article-title":"Cancer modeling meets human organoid technology.","volume":"364","author":"Tuveson","year":"2019","journal-title":"Science."},{"key":"B53","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1038\/s41596-019-0232-9","article-title":"Tumor organoid-T-cell coculture systems.","volume":"15","author":"Cattaneo","year":"2020","journal-title":"Nat Protoc."},{"key":"B54","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.biomaterials.2015.06.002","article-title":"Ex vivo engineered immune organoids for controlled germinal center reactions.","volume":"63","author":"Purwada","year":"2015","journal-title":"Biomaterials."},{"key":"B55","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1038\/nprot.2016.157","article-title":"Immuno-engineered organoids for regulating the kinetics of B-cell development and antibody production.","volume":"12","author":"Purwada","year":"2017","journal-title":"Nat Protoc."},{"key":"B56","doi-asserted-by":"publisher","first-page":"1317","DOI":"10.1056\/NEJMoa066157","article-title":"Telomerase mutations in families with idiopathic pulmonary fibrosis.","volume":"356","author":"Armanios","year":"2007","journal-title":"N Engl J Med."},{"key":"B57","doi-asserted-by":"publisher","first-page":"355","DOI":"10.1016\/j.matbio.2018.03.015","article-title":"Endoplasmic reticulum stress in pulmonary fibrosis.","author":"Burman","year":"2018","journal-title":"Matrix Biol."},{"key":"B58","doi-asserted-by":"publisher","first-page":"322","DOI":"10.1111\/febs.14381","article-title":"Pulmonary endoplasmic reticulum stress-scars, smoke, and suffocation.","volume":"286","author":"Dickens","year":"2019","journal-title":"FEBS J."},{"key":"B59","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1056\/NEJMoa1013660","article-title":"A common MUC5B promoter polymorphism and pulmonary fibrosis.","volume":"364","author":"Seibold","year":"2011","journal-title":"N Engl J Med."},{"key":"B60","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1038\/nature12807","article-title":"Muc5b is required for airway defence.","volume":"505","author":"Roy","year":"2014","journal-title":"Nature."},{"key":"B61","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1016\/j.rmed.2018.12.015","article-title":"Inflammation and immunity in IPF pathogenesis and treatment.","volume":"147","author":"Heukels","year":"2019","journal-title":"Respir Med."},{"key":"B62","doi-asserted-by":"publisher","first-page":"1584","DOI":"10.1378\/chest.12-0110","article-title":"The pulmonary fibrosis-associated MUC5B promoter polymorphism does not influence the development of interstitial pneumonia in systemic sclerosis.","volume":"142","author":"Peljto","year":"2012","journal-title":"Chest."},{"key":"B63","doi-asserted-by":"publisher","first-page":"1173","DOI":"10.1007\/s10067-019-04922-6","article-title":"Defining genetic risk factors for scleroderma-associated interstitial lung disease : IRF5 and STAT4 gene variants are associated with scleroderma while STAT4 is protective against scleroderma-associated interstitial lung disease.","volume":"39","author":"Stock","year":"2020","journal-title":"Clin Rheumatol."},{"key":"B64","doi-asserted-by":"publisher","first-page":"481","DOI":"10.3109\/08820139.2013.789910","article-title":"The CTLA-4 and MCP-1 polymorphisms and susceptibility to systemic sclerosis: a meta-analysis.","volume":"42","author":"Song","year":"2013","journal-title":"Immunol Invest."},{"key":"B65","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1016\/j.stem.2017.08.014","article-title":"Differentiation of human pluripotent stem cells into functional lung alveolar epithelial cells.","volume":"21","author":"Jacob","year":"2017","journal-title":"Cell Stem Cell."},{"key":"B66","doi-asserted-by":"publisher","first-page":"844","DOI":"10.1016\/j.stem.2017.03.001","article-title":"Efficient derivation of functional human airway epithelium from pluripotent stem cells via temporal regulation of wnt signaling.","volume":"20","author":"McCauley","year":"2017","journal-title":"Cell Stem Cell."},{"key":"B67","doi-asserted-by":"publisher","first-page":"3709","DOI":"10.1016\/j.celrep.2019.05.077","article-title":"Modeling of fibrotic lung disease using 3D organoids derived from human pluripotent stem cells.","volume":"27","author":"Strikoudis","year":"2019","journal-title":"Cell Rep."},{"key":"B68","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1016\/j.bbrc.2019.05.187","article-title":"In vitro expansion of endogenous human alveolar epithelial type II cells in fibroblast-free spheroid culture.","volume":"515","author":"Shiraishi","year":"2019","journal-title":"Biochem Biophys Res Commun."},{"key":"B69","first-page":"1112","article-title":"Comparative study of serum surfactant protein-D and KL-6 concentrations in patients with systemic sclerosis as markers for monitoring the activity of pulmonary fibrosis.","volume":"31","author":"Yanaba","year":"2004","journal-title":"J Rheumatol."},{"key":"B70","first-page":"27","article-title":"Surfactant protein D and KL-6 serum levels in systemic sclerosis: correlation with lung and systemic involvement.","volume":"28","author":"Bonella","year":"2011","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis."},{"key":"B71","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.1164\/rccm.2104003","article-title":"Partitioning of alveolar and conducting airway nitric oxide in scleroderma lung disease.","volume":"165","author":"Girgis","year":"2002","journal-title":"Am J Respir Crit Care Med."},{"key":"B72","doi-asserted-by":"publisher","first-page":"1352","DOI":"10.1378\/chest.115.5.1352","article-title":"Exhaled nitric oxide is increased in active fibrosing alveolitis.","volume":"115","author":"Paredi","year":"1999","journal-title":"Chest."},{"key":"B73","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1038\/nrrheum.2010.102","article-title":"Cellular and molecular aspects of vascular dysfunction in systemic sclerosis.","volume":"6","author":"Trojanowska","year":"2010","journal-title":"Nat Rev Rheumatol."},{"key":"B74","doi-asserted-by":"publisher","first-page":"972","DOI":"10.1002\/art.40815","article-title":"Performance of candidate serum biomarkers for systemic sclerosis-associated interstitial lung disease.","volume":"71","author":"Elhai","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"B75","doi-asserted-by":"publisher","first-page":"1871","DOI":"10.1002\/art.40171","article-title":"CCL2 in the circulation predicts long-term progression of interstitial lung disease in patients with early systemic sclerosis: data from two independent cohorts.","volume":"69","author":"Wu","year":"2017","journal-title":"Arthritis Rheumatol."},{"key":"B76","first-page":"228","article-title":"Biomarkers to identify ILD and predict lung function decline in scleroderma lung disease or idiopathic pulmonary fibrosis.","volume":"32","author":"Kennedy","year":"2015","journal-title":"Sarcoidosis Vasc Diffuse Lung Dis."},{"key":"B77","doi-asserted-by":"publisher","first-page":"1064","DOI":"10.1136\/annrheumdis-2011-200837","article-title":"Increased serum levels and tissue expression of matrix metalloproteinase-12 in patients with systemic sclerosis: correlation with severity of skin and pulmonary fibrosis and vascular damage.","volume":"71","author":"Manetti","year":"2012","journal-title":"Ann Rheum Dis."},{"key":"B78","doi-asserted-by":"crossref","first-page":"644","DOI":"10.1164\/rccm.201709-1845OC","article-title":"Reliability and minimal clinically important differences of forced vital capacity: Results from the Scleroderma Lung Studies (SLS-I and SLS-II).","volume":"197","author":"Kafaja","year":"2018","journal-title":"Am J Respir Crit Care Med."},{"key":"B79","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1056\/NEJMc1402401","article-title":"Proteome-wide analysis and CXCL4 as a biomarker in systemic sclerosis.","volume":"370","author":"van Bon","year":"2014","journal-title":"N Engl J Med."},{"key":"B80","doi-asserted-by":"publisher","first-page":"650","DOI":"10.1164\/rccm.201903-0563CI","article-title":"Etiology, risk factors, and biomarkers in systemic sclerosis with interstitial lung disease.","volume":"201","author":"Khanna","year":"2020","journal-title":"Am J Respir Crit Care Med."},{"key":"B81","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1016\/S2213-2600(16)30152-7","article-title":"Mycophenolate mofetil versus oral cyclophosphamide in scleroderma-related interstitial lung disease (SLS II): a randomised controlled, double-blind, parallel group trial.","volume":"4","author":"Tashkin","year":"2016","journal-title":"Lancet Respir Med."},{"key":"B82","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1093\/rheumatology\/key151","article-title":"Ongoing clinical trials and treatment options for patients with systemic sclerosis-associated interstitial lung disease.","volume":"58","author":"Khanna","year":"2019","journal-title":"Rheumatology (Oxford)."},{"key":"B83","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1016\/0268-960X(92)90028-O","article-title":"Mechanisms of action of, and modes of resistance to, alkylating agents used in the treatment of haematological malignancies.","volume":"6","author":"Hall","year":"1992","journal-title":"Blood Rev."},{"key":"B84","doi-asserted-by":"publisher","first-page":"679","DOI":"10.1007\/s00296-020-04515-6","article-title":"Is biological therapy in systemic sclerosis the answer?","volume":"40","author":"Misra","year":"2020","journal-title":"Rheumatol Int."},{"key":"B85","doi-asserted-by":"publisher","first-page":"1968","DOI":"10.1056\/NEJMoa1113354","article-title":"Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis.","volume":"366","author":"Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu","year":"2012","journal-title":"N Engl J Med."},{"key":"B86","doi-asserted-by":"publisher","first-page":"222","DOI":"10.1016\/S0140-6736(09)60551-1","article-title":"Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial.","volume":"374","author":"King","year":"2009","journal-title":"Lancet."},{"key":"B87","doi-asserted-by":"publisher","first-page":"933","DOI":"10.1016\/S2213-2600(14)70232-2","article-title":"Combination therapy: the future of management for idiopathic pulmonary fibrosis?","volume":"2","author":"Wuyts","year":"2014","journal-title":"Lancet Respir Med."},{"key":"B88","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/j.rmed.2017.07.062","article-title":"Therapeutic targets in idiopathic pulmonary fibrosis.","volume":"131","author":"Kolb","year":"2017","journal-title":"Respir Med."},{"key":"B89","doi-asserted-by":"publisher","first-page":"2071","DOI":"10.1056\/NEJMoa1402584","article-title":"Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis.","volume":"370","author":"Richeldi","year":"2014","journal-title":"N Engl J Med."},{"key":"B90","doi-asserted-by":"publisher","first-page":"1434","DOI":"10.1183\/09031936.00174914","article-title":"Mode of action of nintedanib in the treatment of idiopathic pulmonary fibrosis.","volume":"45","author":"Wollin","year":"2015","journal-title":"Eur Respir J."},{"key":"B91","doi-asserted-by":"publisher","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","article-title":"Nintedanib for systemic sclerosis-associated interstitial lung disease.","volume":"380","author":"Distler","year":"2019","journal-title":"N Engl J Med."},{"key":"B92","first-page":"75","article-title":"Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS).","volume":"35","author":"Distler","year":"2017","journal-title":"Clin Exp Rheumatol."},{"key":"B93","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.00383","article-title":"Prominence of IL6, IGF, TLR, and bioenergetics pathway perturbation in lung tissues of scleroderma patients with pulmonary fibrosis.","volume":"11","author":"Renaud","year":"2020","journal-title":"Front Immunol."},{"key":"B94","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1093\/jb\/mvq052","article-title":"Autotaxin\u2013an LPA producing enzyme with diverse functions.","volume":"148","author":"Nakanaga","year":"2010","journal-title":"J Biochem."},{"key":"B95","doi-asserted-by":"publisher","DOI":"10.1186\/1471-2466-14-5","article-title":"Docosatetraenoyl LPA is elevated in exhaled breath condensate in idiopathic pulmonary fibrosis.","volume":"14","author":"Montesi","year":"2014","journal-title":"BMC Pulm Med."},{"key":"B96","doi-asserted-by":"publisher","first-page":"566","DOI":"10.1165\/rcmb.2012-0004OC","article-title":"Pulmonary autotaxin expression contributes to the pathogenesis of pulmonary fibrosis.","volume":"47","author":"Oikonomou","year":"2012","journal-title":"Am J Respir Cell Mol Biol."},{"key":"B97","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1016\/S2213-2600(18)30181-4","article-title":"Safety, tolerability, pharmacokinetics, and pharmacodynamics of GLPG1690, a novel autotaxin inhibitor, to treat idiopathic pulmonary fibrosis (FLORA): a phase 2a randomised placebo-controlled trial.","volume":"6","author":"Maher","year":"2018","journal-title":"Lancet Respir Med."},{"key":"B98","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/S2213-2600(19)30262-0","article-title":"Pamrevlumab, an anti-connective tissue growth factor therapy, for idiopathic pulmonary fibrosis (PRAISE): a phase 2, randomised, double-blind, placebo-controlled trial.","volume":"8","author":"Richeldi","year":"2020","journal-title":"Lancet Respir Med."},{"key":"B99","doi-asserted-by":"publisher","first-page":"2299","DOI":"10.1001\/jama.2018.6129","article-title":"Effect of recombinant human pentraxin 2 vs placebo on change in forced vital capacity in patients with idiopathic pulmonary fibrosis: a randomized clinical trial.","volume":"319","author":"Raghu","year":"2018","journal-title":"JAMA."},{"key":"B100","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-019-0980-7","article-title":"Interstitial lung disease associated with systemic sclerosis (SSc-ILD).","volume":"20","author":"Cottin","year":"2019","journal-title":"Respir Res."},{"key":"B101","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-019-11867-6","article-title":"Patient-derived lung cancer organoids as in vitro cancer models for therapeutic screening.","volume":"10","author":"Kim","year":"2019","journal-title":"Nat Commun."},{"key":"B102","doi-asserted-by":"publisher","first-page":"1701","DOI":"10.1016\/j.celrep.2019.01.068","article-title":"Rectal organoids enable personalized treatment of cystic fibrosis.","volume":"26","author":"Berkers","year":"2019","journal-title":"Cell Rep"},{"key":"B103","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1164\/rccm.201508-1638OC","article-title":"Stretch-induced activation of transforming growth factor-beta1 in pulmonary fibrosis.","volume":"194","author":"Froese","year":"2016","journal-title":"Am J Respir Crit Care Med."},{"key":"B104","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.addr.2018.07.005","article-title":"Stem cell-based lung-on-chips: the best of both worlds?","volume":"140","author":"Nawroth","year":"2019","journal-title":"Adv Drug Deliv Rev."},{"key":"B105","doi-asserted-by":"publisher","first-page":"1379","DOI":"10.1136\/annrheumdis-2018-214865","article-title":"Single-cell analysis reveals fibroblast heterogeneity and myofibroblasts in systemic sclerosis-associated interstitial lung disease.","volume":"78","author":"Valenzi","year":"2019","journal-title":"Ann Rheum Dis."},{"key":"B106","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.02534","article-title":"40-marker panel for high dimensional characterization of cancer immune microenvironments by imaging mass cytometry.","volume":"10","author":"Ijsselsteijn","year":"2019","journal-title":"Front Immunol."},{"key":"B107","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1002\/eji.201948129","article-title":"Cytometry by time of flight identifies distinct signatures in patients with systemic sclerosis, systemic lupus erythematosus and Sjogrens syndrome.","volume":"50","author":"Van Der Kroef","year":"2020","journal-title":"Eur J Immunol."}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fimmu.2020.01990\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,4]],"date-time":"2020-09-04T13:52:20Z","timestamp":1599227540000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/article\/10.3389\/fimmu.2020.01990\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9,4]]},"references-count":107,"alternative-id":["10.3389\/fimmu.2020.01990"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2020.01990","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,9,4]]},"article-number":"1990"}}