{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T19:35:56Z","timestamp":1785612956793,"version":"3.56.0"},"reference-count":141,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2020,12,28]],"date-time":"2020-12-28T00:00:00Z","timestamp":1609113600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Swedish Research Council in Medicine and Health","award":["11550, 2016-01190"],"award-info":[{"award-number":["11550, 2016-01190"]}]},{"name":"VR3R","award":["2016-01190"],"award-info":[{"award-number":["2016-01190"]}]},{"name":"Swedish Heart-Lung Foundation","award":["20140293"],"award-info":[{"award-number":["20140293"]}]},{"DOI":"10.13039\/501100001729","name":"Swedish foundation for Strategic Research","doi-asserted-by":"publisher","award":["SBE13-0130"],"award-info":[{"award-number":["SBE13-0130"]}],"id":[{"id":"10.13039\/501100001729","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Royal Physiographic Society of Lund","award":["-"],"award-info":[{"award-number":["-"]}]},{"name":"the Olle Engkvist Foundation","award":["-"],"award-info":[{"award-number":["-"]}]},{"DOI":"10.13039\/501100003173","name":"the Crafoord Foundation","doi-asserted-by":"publisher","award":["-"],"award-info":[{"award-number":["-"]}],"id":[{"id":"10.13039\/501100003173","id-type":"DOI","asserted-by":"publisher"}]},{"name":"the Greta and John Kock Foundation","award":["-"],"award-info":[{"award-number":["-"]}]},{"name":"the Alfred \u00d6sterlund Foundation","award":["-"],"award-info":[{"award-number":["-"]}]},{"name":"the \u00c5ke och Inger Bergkvist foundation","award":["-"],"award-info":[{"award-number":["-"]}]},{"name":"the Medical Faculty of Lund University","award":["-"],"award-info":[{"award-number":["-"]}]},{"DOI":"10.13039\/501100001034","name":"ALF","doi-asserted-by":"publisher","award":["-"],"award-info":[{"award-number":["-"]}],"id":[{"id":"10.13039\/501100001034","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Interstitial lung disease (ILD) encompasses a heterogeneous group of more than 200 conditions, of which primarily idiopathic pulmonary fibrosis (IPF), idiopathic nonspecific interstitial pneumonia, hypersensitivity pneumonitis, ILD associated with autoimmune diseases and sarcoidosis may present a progressive fibrosing (PF) phenotype. Despite different aetiology and histopathological patterns, the PF-ILDs have similarities regarding disease mechanisms with self-sustaining fibrosis, which suggests that the diseases may share common pathogenetic pathways. Previous studies show an enhanced activation of serotonergic signaling in pulmonary fibrosis, and the serotonin (5-HT)2 receptors have been implicated to have important roles in observed profibrotic actions. Our research findings in support by others, demonstrate antifibrotic effects with 5-HT2B receptor antagonists, alleviating several key events common for the fibrotic diseases such as myofibroblast differentiation and connective tissue deposition. In this review, we will address the potential role of 5-HT and in particular the 5-HT2B receptors in three PF-ILDs: ILD associated with systemic sclerosis (SSc-ILD), ILD associated with rheumatoid arthritis (RA-ILD) and IPF. Highlighting the converging pathways in these diseases discloses the 5-HT2B receptor as a potential disease target for PF-ILDs, which today have an urgent unmet need for therapeutic strategies.<\/jats:p>","DOI":"10.3390\/ijms22010225","type":"journal-article","created":{"date-parts":[[2020,12,28]],"date-time":"2020-12-28T20:03:03Z","timestamp":1609185783000},"page":"225","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Pathological Insight into 5-HT2B Receptor Activation in Fibrosing Interstitial Lung Diseases"],"prefix":"10.3390","volume":"22","author":[{"given":"Anna","family":"L\u00f6fdahl","sequence":"first","affiliation":[{"name":"Lung Biology, Department of Experimental Medical Science, Lund University, BMC C12, 22184 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00f6ran","family":"Tornling","sequence":"additional","affiliation":[{"name":"AnaMar AB, Medicon Village, Scheeletorget 1, 22381 Lund, Sweden"},{"name":"Respiratory Medicine Division, Department of Medicine Solna, Karolinska Institutet, 17177 Stockholm, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny","family":"Wig\u00e9n","sequence":"additional","affiliation":[{"name":"Lung Biology, Department of Experimental Medical Science, Lund University, BMC C12, 22184 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7586-3896","authenticated-orcid":false,"given":"Anna-Karin","family":"Larsson-Callerfelt","sequence":"additional","affiliation":[{"name":"Lung Biology, Department of Experimental Medical Science, Lund University, BMC C12, 22184 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Wengl\u00e9n","sequence":"additional","affiliation":[{"name":"AnaMar AB, Medicon Village, Scheeletorget 1, 22381 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gunilla","family":"Westergren-Thorsson","sequence":"additional","affiliation":[{"name":"Lung Biology, Department of Experimental Medical Science, Lund University, BMC C12, 22184 Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,12,28]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"180076","DOI":"10.1183\/16000617.0076-2018","article-title":"Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases","volume":"27","author":"Cottin","year":"2018","journal-title":"Eur. Respir. Rev."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"180100","DOI":"10.1183\/16000617.0100-2018","article-title":"Fibrosing interstitial lung diseases: Knowns and unknowns","volume":"28","author":"Cottin","year":"2019","journal-title":"Eur. Respir. Rev."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","article-title":"Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS\/ERS\/JRS\/ALAT Clinical Practice Guideline","volume":"198","author":"Raghu","year":"2018","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1183\/09031936.00185114","article-title":"Global incidence and mortality of idiopathic pulmonary fibrosis: A systematic review","volume":"46","author":"Hutchinson","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1164\/rccm.2009-040GL","article-title":"An Official ATS\/ERS\/JRS\/ALAT Statement: Idiopathic Pulmonary Fibrosis: Evidence-based Guidelines for Diagnosis and Management","volume":"183","author":"Raghu","year":"2011","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Balestro, E., Calabrese, F., Turato, G., Lunardi, F., Bazzan, E., Marulli, G., Biondini, D., Rossi, E., Sanduzzi, A., and Rea, F. (2016). Immune Inflammation and Disease Progression in Idiopathic Pulmonary Fibrosis. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0154516"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1146\/annurev-med-041317-102715","article-title":"Progress in Understanding and Treating Idiopathic Pulmonary Fibrosis","volume":"70","author":"Kropski","year":"2019","journal-title":"Annu. Rev. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"17074","DOI":"10.1038\/nrdp.2017.74","article-title":"Idiopathic pulmonary fibrosis","volume":"3","author":"Martinez","year":"2017","journal-title":"Nat. Rev. Dis. Prim."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"190158","DOI":"10.1183\/16000617.0158-2019","article-title":"Prognosis of idiopathic pulmonary fibrosis without anti-fibrotic therapy: A systematic review","volume":"29","author":"Khor","year":"2020","journal-title":"Eur. Respir. Rev."},{"key":"ref_10","first-page":"401","article-title":"Acute Exacerbation of Idiopathic Pulmonary Fibrosis","volume":"194","author":"Lee","year":"2018","journal-title":"Pulm. Funct. Test."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1038\/nrrheum.2016.223","article-title":"Systemic sclerosis in 2016: Dermal white adipose tissue implicated in SSc pathogenesis","volume":"13","author":"Varga","year":"2017","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"248","DOI":"10.3389\/fmed.2019.00248","article-title":"Systemic Sclerosis Associated Interstitial Lung Disease: New Directions in Disease Management","volume":"6","author":"Mirsaeidi","year":"2019","journal-title":"Front. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2103","DOI":"10.1007\/s10067-018-4182-3","article-title":"The incidence, prevalence, and survival of systemic sclerosis in the UK Clinical Practice Research Datalink","volume":"37","author":"Royle","year":"2018","journal-title":"Clin. Rheumatol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1136\/annrheumdis-2014-206386","article-title":"A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: A EUSTAR prospective study","volume":"75","author":"Elhai","year":"2014","journal-title":"Ann. Rheum. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1685","DOI":"10.1016\/S0140-6736(17)30933-9","article-title":"Systemic sclerosis","volume":"390","author":"Denton","year":"2017","journal-title":"Lancet"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1897","DOI":"10.1136\/annrheumdis-2017-211448","article-title":"Mapping and predicting mortality from systemic sclerosis","volume":"76","author":"Elhai","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1902026","DOI":"10.1183\/13993003.02026-2019","article-title":"Predictors of progression in systemic sclerosis patients with interstitial lung disease","volume":"55","author":"Distler","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1892","DOI":"10.1002\/art.41415","article-title":"Performance Characteristics of Pulmonary Function Tests for the Detection of Interstitial Lung Disease in Adults With Early Diffuse Cutaneous Systemic Sclerosis","volume":"72","author":"Bernstein","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Vacchi, C., Sebastiani, M., Cassone, G., Cerri, S., Della Casa, G., Salvarani, C., and Manfredi, A. (2020). Therapeutic Options for the Treatment of Interstitial Lung Disease Related to Connective Tissue Diseases. A Narrative Review. J. Clin. Med., 9.","DOI":"10.3390\/jcm9020407"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1136\/ard.2006.062901","article-title":"Clinical risk assessment of organ manifestations in systemic sclerosis: A report from the EULAR Scleroderma Trials And Research group database","volume":"66","author":"Walker","year":"2007","journal-title":"Ann. Rheum. Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1007\/s40744-017-0071-5","article-title":"Practical Management of Respiratory Comorbidities in Patients with Rheumatoid Arthritis","volume":"4","author":"Bluett","year":"2017","journal-title":"Rheumatol. Ther."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1097\/MCP.0000000000000600","article-title":"New trajectories in the treatment of interstitial lung disease: Treat the disease or treat the underlying pattern?","volume":"25","author":"Morisset","year":"2019","journal-title":"Curr. Opin. Pulm. Med."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"360","DOI":"10.3899\/jrheum.171315","article-title":"Rheumatoid Arthritis\u2013Interstitial Lung Disease in the United States: Prevalence, Incidence, and Healthcare Costs and Mortality","volume":"46","author":"Raimundo","year":"2018","journal-title":"J. Rheumatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"277","DOI":"10.2174\/1573397115666190116102451","article-title":"Reinterpreting Evidence of Rheumatoid Arthritis-Associated Interstitial Lung Disease to Understand Etiology","volume":"15","author":"Wu","year":"2019","journal-title":"Curr. Rheumatol. Rev."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1016\/j.ccm.2019.05.003","article-title":"Thoracic Manifestations of Rheumatoid Arthritis","volume":"40","author":"Esposito","year":"2019","journal-title":"Clin. Chest Med."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.1002\/art.40904","article-title":"Rheumatoid Arthritis Disease Activity Predicting Incident Clinically Apparent Rheumatoid Arthritis\u2013Associated Interstitial Lung Disease: A Prospective Cohort Study","volume":"71","author":"Sparks","year":"2019","journal-title":"Arthritis Rheumatol."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Idiopathic Pulmonary Fibrosis Clinical Research Network (2012). Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N. Engl. J. Med., 366, 1968\u20131977.","DOI":"10.1056\/NEJMoa1113354"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1007\/s00296-010-1697-6","article-title":"Corticosteroids and the risk of scleroderma renal crisis: A systematic review","volume":"32","author":"Trang","year":"2010","journal-title":"Rheumatol. Int."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"190021","DOI":"10.1183\/16000617.0021-2019","article-title":"The therapy of idiopathic pulmonary fibrosis: What is next?","volume":"28","author":"Somogyi","year":"2019","journal-title":"Eur. Respir. Rev."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"8341426-11","DOI":"10.1155\/2020\/8341426","article-title":"Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome","volume":"2020","author":"Clement","year":"2020","journal-title":"J. Oncol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"2836","DOI":"10.1161\/01.CIR.102.23.2836","article-title":"Evidence for Possible Involvement of 5-HT 2B Receptors in the Cardiac Valvulopathy Associated With Fenfluramine and Other Serotonergic Medications","volume":"102","author":"Rothman","year":"2000","journal-title":"Circulation"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1517\/14740330902931524","article-title":"Serotonergic drugs and valvular heart disease","volume":"8","author":"Rothman","year":"2009","journal-title":"Expert. Opin. Drug Saf."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.pharmthera.2011.03.008","article-title":"Serotonin receptors and heart valve disease\u2014It was meant 2B","volume":"132","author":"Hutcheson","year":"2011","journal-title":"Pharmacol. Ther."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"e12873","DOI":"10.14814\/phy2.12873","article-title":"5-HT2B receptor antagonists attenuate myofibroblast differentiation and subsequent fibrotic responses in vitro and in vivo","volume":"4","author":"Holst","year":"2016","journal-title":"Physiol. Rep."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1244","DOI":"10.1016\/j.pharep.2016.08.005","article-title":"The antifibrotic effects of alveolar macrophages 5-HT2C receptors blockade on bleomycin-induced pulmonary fibrosis in rats","volume":"68","author":"Elaidy","year":"2016","journal-title":"Pharmacol. Rep."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1183\/09031936.00126907","article-title":"Modulation of bleomycin-induced lung fibrosis by serotonin receptor antagonists in mice","volume":"32","author":"Fabre","year":"2008","journal-title":"Eur. Respir. J."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.ejphar.2017.08.014","article-title":"5-HT7 receptor antagonism (SB-269970) attenuates bleomycin-induced pulmonary fibrosis in rats via downregulating oxidative burden and inflammatory cascades and ameliorating collagen deposition: Comparison to terguride","volume":"814","author":"Tawfik","year":"2017","journal-title":"Eur. J. Pharmacol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1084\/jem.20101629","article-title":"Platelet-derived serotonin links vascular disease and tissue fibrosis","volume":"208","author":"Dees","year":"2011","journal-title":"J. Exp. Med."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"949","DOI":"10.1136\/thx.2009.134353","article-title":"Increased expression of 5-hydroxytryptamine2A\/B receptors in idiopathic pulmonary fibrosis: A rationale for therapeutic intervention","volume":"65","author":"Dumitrascu","year":"2010","journal-title":"Thorax"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1668","DOI":"10.1038\/nm.2490","article-title":"Stimulating healthy tissue regeneration by targeting the 5-HT2B receptor in chronic liver disease","volume":"17","author":"Ebrahimkhani","year":"2011","journal-title":"Nat. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"3663","DOI":"10.1007\/s00018-018-2880-2","article-title":"Tryptophan metabolites kynurenine and serotonin regulate fibroblast activation and fibrosis","volume":"75","author":"Dolivo","year":"2018","journal-title":"Cell. Mol. Life Sci."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"L185","DOI":"10.1152\/ajplung.00167.2005","article-title":"Mechanical stretch-induced serotonin release from pulmonary neuroendocrine cells: Implications for lung development","volume":"290","author":"Pan","year":"2006","journal-title":"Am. J. Physiol. Cell. Mol. Physiol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1016\/j.jaci.2006.09.003","article-title":"Human mast cells are capable of serotonin synthesis and release","volume":"119","author":"Brown","year":"2007","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1002\/wmts.50","article-title":"Serotonergic signaling: Multiple effectors and pleiotropic effects","volume":"1","author":"Masson","year":"2012","journal-title":"WIREs Membr. Transp. Signal."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1038\/nrrheum.2009.26","article-title":"Transforming growth factor beta as a therapeutic target in systemic sclerosis","volume":"5","author":"Varga","year":"2009","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1046\/j.1523-1747.1998.00073.x","article-title":"Increased expression of TGF-beta receptors by scleroderma fibroblasts: Evidence for contribution of autocrine TGF-beta signaling to scleroderma phenotype","volume":"110","author":"Kawakami","year":"1998","journal-title":"J. Invest. Dermatol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"L1327","DOI":"10.1152\/ajplung.2001.280.6.L1327","article-title":"Proteoglycans decorin and biglycan differentially modulate TGF-beta-mediated fibrotic responses in the lung","volume":"280","author":"Kolb","year":"2001","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"4857","DOI":"10.1242\/jcs.00808","article-title":"Biglycan and decorin induce morphological and cytoskeletal changes involving signalling by the small GTPases RhoA and Rac1 resulting in lung fibroblast migration","volume":"116","author":"Tufvesson","year":"2003","journal-title":"J. Cell Sci."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1113","DOI":"10.1016\/j.ajpath.2018.01.006","article-title":"Effects of 5-Hydroxytryptamine Class 2 Receptor Antagonists on Bronchoconstriction and Pulmonary Remodeling Processes","volume":"188","year":"2018","journal-title":"Am. J. Pathol."},{"key":"ref_50","first-page":"PA2421","article-title":"An orally available, highly selective 5-hydroxytryptamine 2B (5-HT2B) receptor antagonist ameliorating pulmonary and dermal fibrosis","volume":"54","author":"Arozenius","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Wengl\u00e9n, C., Pettersson, A.H., Ekstr\u00f6m, L., and An, G. (2018). Orally Available Highly Selective 5-Hydroxytryptamine 2B Receptor Antagonist Ameliorating Pulmonary and Dermal Fibrosis in Preclinical Models of Systemic Sclerosis [abstract]. Arthritis Rheumatol., 70.","DOI":"10.1183\/13993003.congress-2019.PA2421"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1002\/art.22328","article-title":"Postnatal induction of transforming growth factor beta signaling in fibroblasts of mice recapitulates clinical, histologic, and biochemical features of scleroderma","volume":"56","author":"Sonnylal","year":"2007","journal-title":"Arthritis Rheum."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1002\/jcp.22783","article-title":"PAI-1 in tissue fibrosis","volume":"227","author":"Ghosh","year":"2012","journal-title":"J. Cell. Physiol."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"9428","DOI":"10.1074\/jbc.M114.601815","article-title":"Plasminogen Activator Inhibitor-1 Suppresses Profibrotic Responses in Fibroblasts from Fibrotic Lungs","volume":"290","author":"Marudamuthu","year":"2015","journal-title":"J. Biol. Chem."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.thromres.2012.09.003","article-title":"Plasminogen activator inhibitor-1 promotes the proliferation and inhibits the apoptosis of pulmonary fibroblasts by Ca2+ signaling","volume":"131","author":"Zhang","year":"2013","journal-title":"Thromb. Res."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1046\/j.1524-475X.2002.t01-1-10608.x","article-title":"Differential expression of urokinase-type plasminogen activator and plasminogen activator inhibitor-1 in early and late gestational mouse skin and skin wounds","volume":"10","author":"Huang","year":"2002","journal-title":"Wound Repair Regen."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1002\/art.39443","article-title":"Resolution of Skin Fibrosis by Neutralization of the Antifibrinolytic Function of Plasminogen Activator Inhibitor 1","volume":"68","author":"Lemaire","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"L463","DOI":"10.1152\/ajplung.00099.2011","article-title":"Regulation of alveolar epithelial cell apoptosis and pulmonary fibrosis by coordinate expression of components of the fibrinolytic system","volume":"302","author":"Bhandary","year":"2012","journal-title":"Am. J. Physiol. Cell. Mol. Physiol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"173","DOI":"10.3389\/fphar.2013.00173","article-title":"The epithelium in idiopathic pulmonary fibrosis: Breaking the barrier","volume":"4","author":"Camelo","year":"2014","journal-title":"Front. Pharmacol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"517","DOI":"10.1056\/NEJMra003200","article-title":"Medical progress: Idiopathic pulmonary fibrosis","volume":"345","author":"Gross","year":"2001","journal-title":"N. Engl. J. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"591","DOI":"10.5858\/arpa.2011-0511-OA","article-title":"Idiopathic Pulmonary Fibrosis May Be a Disease of Recurrent, Tractional Injury to the Periphery of the Aging Lung: A Unifying Hypothesis Regarding Etiology and Pathogenesis","volume":"136","author":"Leslie","year":"2012","journal-title":"Arch. Pathol. Lab. Med."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1016\/S0002-9440(10)63674-7","article-title":"Reduction in Fibrotic Tissue Formation in Mice Genetically Deficient in Plasminogen Activator Inhibitor-1","volume":"163","author":"Sisson","year":"2003","journal-title":"Am. J. Pathol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"2128","DOI":"10.1111\/1756-185X.13386","article-title":"5-HT2 and 5-HT2B antagonists attenuate pro-fibrotic phenotype in human adult dermal fibroblasts by blocking TGF-beta1 induced non-canonical signaling pathways including STAT3: Implications for fibrotic diseases like scleroderma","volume":"21","author":"Chaturvedi","year":"2018","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1016\/j.taap.2017.06.012","article-title":"Modulation of TGF-beta\/Smad and ERK signaling pathways mediates the anti-fibrotic effect of mirtazapine in mice","volume":"329","author":"Wadie","year":"2017","journal-title":"Toxicol. Appl. Pharmacol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1016\/j.yjmcc.2012.08.012","article-title":"5-HT(2B) antagonism arrests non-canonical TGF-beta1-induced valvular myofibroblast differentiation","volume":"53","author":"Hutcheson","year":"2012","journal-title":"J. Mol. Cell. Cardiol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41467-017-01236-6","article-title":"Activation of STAT3 integrates common profibrotic pathways to promote fibroblast activation and tissue fibrosis","volume":"8","author":"Chakraborty","year":"2017","journal-title":"Nat. Commun."},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Fong, F., Xian, J., Demer, L.L., and Tintut, Y. (2020). Serotonin receptor type 2B activation augments TNF-\u03b1-induced matrix mineralization in murine valvular interstitial cells. J. Cell. Biochem.","DOI":"10.1002\/jcb.29847"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1093\/cvr\/cvp205","article-title":"Serotonin enhances platelet procoagulant properties and their activation induced during platelet tissue factor uptake","volume":"84","author":"White","year":"2009","journal-title":"Cardiovasc. Res."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"917","DOI":"10.1111\/j.1476-5381.1992.tb14435.x","article-title":"Potentiation of ADP-induced aggregation in human platelet-rich plasma by 5-hydroxytryptamine and adrenaline","volume":"106","author":"Vanags","year":"1992","journal-title":"Br. J. Pharmacol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/j.transci.2004.01.004","article-title":"Platelet gel for healing cutaneous chronic wounds","volume":"30","author":"Crovetti","year":"2004","journal-title":"Transfus. Apher. Sci."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s12929-017-0324-1","article-title":"Treatment of chronic non-healing ulcers using autologous platelet rich plasma: A case series","volume":"24","author":"Suthar","year":"2017","journal-title":"J. Biomed. Sci."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1083\/jcb.200105058","article-title":"Activated platelets mediate inflammatory signaling by regulated interleukin 1beta synthesis","volume":"154","author":"Lindemann","year":"2001","journal-title":"J. Cell Biol."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1160\/TH08-06-0406","article-title":"Dynamics of platelet mobilisation into lungs in response to 5-hydroxytryptamine (serotonin) in mice","volume":"102","author":"Yu","year":"2009","journal-title":"Thromb. Haemost."},{"key":"ref_74","first-page":"105","article-title":"The lung is a site of platelet biogenesis and a reservoir for haematopoietic progenitors","volume":"544","author":"Caudrillier","year":"2017","journal-title":"Nat. Cell Biol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1007\/s10517-014-2682-y","article-title":"Response of Hemopoietic, Progenitor, and Multipotent Mesenchymal Stromal Cells to Administration of Ketanserin during Pulmonary Fibrosis","volume":"158","author":"Dygai","year":"2014","journal-title":"Bull. Exp. Biol. Med."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1900100","DOI":"10.1183\/13993003.00100-2019","article-title":"Vascular permeability in the fibrotic lung","volume":"56","author":"Probst","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1989","DOI":"10.1056\/NEJMra0806188","article-title":"Scleroderma","volume":"360","author":"Gabrielli","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1007\/s11926-019-0815-z","article-title":"Platelets in Systemic Sclerosis: The Missing Link Connecting Vasculopathy, Autoimmunity, and Fibrosis?","volume":"21","author":"Ntelis","year":"2019","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"13","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":"ref_80","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1183\/09059180.00008014","article-title":"Rheumatoid arthritis-associated lung disease","volume":"24","author":"Shaw","year":"2015","journal-title":"Eur. Respir. Rev."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1016\/j.ajpath.2015.11.018","article-title":"Serotonin Is Involved in Autoimmune Arthritis through Th17 Immunity and Bone Resorption","volume":"186","author":"Coman","year":"2016","journal-title":"Am. J. Pathol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1038\/nrrheum.2012.118","article-title":"Platelets: Active players in the pathogenesis of arthritis and SLE","volume":"8","author":"Boilard","year":"2012","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"601S","DOI":"10.1378\/chest.128.6_suppl.601S","article-title":"Abnormal Vascular Phenotypes in Patients With Idiopathic Pulmonary Fibrosis and Secondary Pulmonary Hypertension","volume":"128","author":"Gagermeier","year":"2005","journal-title":"Chest"},{"key":"ref_84","first-page":"225","article-title":"Precapillary Systemic-pulmonary Anastomoses","volume":"18","year":"2008","journal-title":"Thorax"},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1093\/qjmed\/hcu012","article-title":"Vascular remodelling in the pathogenesis of idiopathic pulmonary fibrosis","volume":"107","author":"Barratt","year":"2014","journal-title":"QJM"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1655","DOI":"10.1056\/NEJMra035488","article-title":"Pulmonary arterial hypertension","volume":"351","author":"Farber","year":"2004","journal-title":"N. Engl. J. Med."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1183\/09031936.00171307","article-title":"Pulmonary hypertension in interstitial lung disease","volume":"31","author":"Behr","year":"2008","journal-title":"Eur. Respir. J."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"540","DOI":"10.1016\/S0761-8425(06)71883-6","article-title":"055 Cross-talk between endothelial and smooth muscle cells in pulmonary hypertension: Critical role for serotonin-induced smooth muscle hyperplasia","volume":"23","author":"Eddahibi","year":"2006","journal-title":"Rev. Mal. Respir."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1124\/jpet.116.237933","article-title":"Tryptophan hydroxylase 1 Inhibition Impacts Pulmonary Vascular Remodeling in Two Rat Models of Pulmonary Hypertension","volume":"360","author":"Aiello","year":"2016","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"ref_90","doi-asserted-by":"crossref","unstructured":"West, J., Carrier, E.J., Bloodworth, N.C., Schroer, A.K., Chen, P., Ryzhova, L.M., Gladson, S., Shay, S., Hutcheson, J.D., and Merryman, W.D. (2016). Serotonin 2B Receptor Antagonism Prevents Heritable Pulmonary Arterial Hypertension. PLoS ONE, 11.","DOI":"10.1371\/journal.pone.0148657"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1038\/nm764","article-title":"Function of the serotonin 5-hydroxytryptamine 2B receptor in pulmonary hypertension","volume":"8","author":"Launay","year":"2002","journal-title":"Nat. Med."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"186","DOI":"10.3389\/fimmu.2020.00186","article-title":"Serotonin: A Potent Immune Cell Modulator in Autoimmune Diseases","volume":"11","author":"Wan","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1111\/apha.12430","article-title":"The role of serotonin and its receptors in activation of immune responses and inflammation","volume":"213","author":"Shajib","year":"2015","journal-title":"Acta Physiol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1007\/978-3-319-07320-0_9","article-title":"Serotonin modulation of macrophage polarization: Inflammation and beyond","volume":"824","author":"Corbi","year":"2014","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1093\/intimm\/dxh242","article-title":"5-Hydroxytryptamine modulates cytokine and chemokine production in LPS-primed human monocytes via stimulation of different 5-HTR subtypes","volume":"17","author":"Panther","year":"2005","journal-title":"Int. Immunol."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2018\/9372436","article-title":"Serotonin Exhibits Accelerated Bleomycin-Induced Pulmonary Fibrosis through TPH1 Knockout Mouse Experiments","volume":"2018","author":"Zhang","year":"2018","journal-title":"Mediat. Inflamm."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1186\/s12950-016-0127-2","article-title":"A human and animal model-based approach to investigating the anti-inflammatory profile and potential of the 5-HT2B receptor antagonist AM1030","volume":"13","author":"Palmqvist","year":"2016","journal-title":"J. Inflamm."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"316","DOI":"10.1124\/jpet.108.143461","article-title":"Serotonin 5-Hydroxytryptamine2A Receptor Activation Suppresses Tumor Necrosis Factor-\u03b1-Induced Inflammation with Extraordinary Potency","volume":"327","author":"Yu","year":"2008","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.ejphar.2012.11.039","article-title":"Modulation of antigen-induced responses by serotonin and prostaglandin E2 via EP1 and EP4 receptors in the peripheral rat lung","volume":"699","author":"Lex","year":"2013","journal-title":"Eur. J. Pharmacol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1016\/j.psychres.2004.01.014","article-title":"Effects of serotonin and serotonergic agonists and antagonists on the production of tumor necrosis factor \u03b1 and interleukin-6","volume":"134","author":"Kubera","year":"2005","journal-title":"Psychiatry Res."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1111","DOI":"10.1136\/ard.2007.074948","article-title":"Genetic variations in the serotonin 5-HT2A receptor gene (HTR2A) are associated with rheumatoid arthritis","volume":"67","author":"Kling","year":"2008","journal-title":"Ann. Rheum. Dis."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1080\/09629350290000069","article-title":"Blood serotonin and joint pain in seropositive versus seronegative rheumatoid arthritis","volume":"11","author":"Kopp","year":"2002","journal-title":"Mediat. Inflamm."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/S0003-9969(99)00026-6","article-title":"Serotonin in an antigen-induced arthritis of the rabbit temporomandibular joint","volume":"44","author":"Tominaga","year":"1999","journal-title":"Arch. Oral Biol."},{"key":"ref_104","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1016\/j.drudis.2012.02.009","article-title":"Impact of 5-HT3 receptor antagonists on peripheral and central diseases","volume":"17","author":"Fakhfouri","year":"2012","journal-title":"Drug Discov. Today"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1182\/blood-2012-02-413047","article-title":"Platelets can enhance vascular permeability","volume":"120","author":"Cloutier","year":"2012","journal-title":"Blood"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/S0304-3959(97)00022-5","article-title":"Pain and synovial fluid concentration of serotonin in arthritic temporomandibular joints","volume":"72","author":"Alstergren","year":"1997","journal-title":"Pain"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1007\/s00011-003-1224-2","article-title":"Leukotrienes mediate 5-hydroxytryptamine-induced plasma extravasation in the rat knee joint via CysLT-type receptors","volume":"53","author":"Wang","year":"2004","journal-title":"Inflamm. Res."},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1189\/jlb.0906544","article-title":"Nicotine and serotonin in immune regulation and inflammatory processes: A perspective","volume":"81","author":"Changeux","year":"2007","journal-title":"J. Leukoc. Biol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.1007\/s00296-008-0564-1","article-title":"Serotonin mediates PGE2 overexpression through 5-HT2A and 5-HT3 receptor subtypes in serum-free tissue culture of macrophage-like synovial cells","volume":"28","author":"Seidel","year":"2008","journal-title":"Rheumatol. Int."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1002\/art.1780260413","article-title":"Serotonin content of platelets in inflammatory rheumatic diseases","volume":"26","author":"Zeller","year":"1983","journal-title":"Arthritis Rheum."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1186\/s13075-015-0848-2","article-title":"Inhibition of angiogenesis by platelets in systemic sclerosis patients","volume":"17","author":"Hirigoyen","year":"2015","journal-title":"Arthritis Res."},{"key":"ref_112","first-page":"280","article-title":"Rheumatoid Arthritis-Associated Interstitial Lung Disease and Idiopathic Pulmonary Fibrosis: Shared Mechanistic and Phenotypic Traits Suggest Overlapping Disease Mechanisms","volume":"67","author":"Paulin","year":"2015","journal-title":"Rev. Invest. Clin."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1183\/09059180.00005512","article-title":"Scleroderma lung disease","volume":"22","author":"Solomon","year":"2013","journal-title":"Eur. Respir. Rev."},{"key":"ref_114","first-page":"1","article-title":"Fibroblast paracrine TNF-\u03b1 signaling elevates integrin A5 expression in idiopathic pulmonary fibrosis (IPF)","volume":"18","author":"Shochet","year":"2017","journal-title":"Respir. Res."},{"key":"ref_115","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1007\/s10456-012-9306-9","article-title":"Imbalance of circulating endothelial cells and progenitors in idiopathic pulmonary fibrosis","volume":"16","author":"Smadja","year":"2012","journal-title":"Angiogenesis"},{"key":"ref_116","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1007\/s12015-017-9778-5","article-title":"Endothelial Microparticles are Associated to Pathogenesis of Idiopathic Pulmonary Fibrosis","volume":"14","author":"Bacha","year":"2018","journal-title":"Stem Cell Rev. Rep."},{"key":"ref_117","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/0167-5273(88)90238-0","article-title":"Plasma free and intraplatelet serotonin in patients with Raynaud\u2019s phenomenon","volume":"19","author":"Biondi","year":"1988","journal-title":"Int. J. Cardiol."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.mvr.2017.04.004","article-title":"Detection of early endothelial damage in patients with Raynaud\u2019s phenomenon","volume":"113","author":"Gualtierotti","year":"2017","journal-title":"Microvasc. Res."},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"30059","DOI":"10.1038\/srep30059","article-title":"Serotonin biosynthesis as a predictive marker of serotonin pharmacodynamics and disease-induced dysregulation","volume":"6","author":"Welford","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"4782","DOI":"10.1111\/febs.12444","article-title":"The expanding roles of 1-methyl-tryptophan (1-MT): In addition to inhibiting kynurenine production, 1-MT activates the synthesis of melatonin in skin cells","volume":"280","author":"Moreno","year":"2013","journal-title":"FEBS J."},{"key":"ref_121","first-page":"G254","article-title":"Function, expression, and characterization of the serotonin transporter in the native human intestine","volume":"294","author":"Gill","year":"2008","journal-title":"Am. J. Physiol. Liver Physiol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"S19","DOI":"10.1186\/1755-1536-5-S1-S19","article-title":"Molecular targets for therapy in systemic sclerosis","volume":"5","author":"Iwamoto","year":"2012","journal-title":"Fibrogenes. Tissue Repair"},{"key":"ref_123","first-page":"65","article-title":"Animal models of systemic sclerosis: Their utility and limitations","volume":"6","author":"Artlett","year":"2014","journal-title":"Open Access Rheumatol. Res. Rev."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"2831","DOI":"10.1002\/art.27647","article-title":"Animal models of systemic sclerosis: Prospects and limitations","volume":"62","author":"Beyer","year":"2010","journal-title":"Arthritis Rheum."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1007\/s11926-015-0553-9","article-title":"Critical Appraisal of the Utility and Limitations of Animal Models of Scleroderma","volume":"18","author":"Tsujino","year":"2016","journal-title":"Curr. Rheumatol. Rep."},{"key":"ref_126","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/S2213-2600(16)30325-3","article-title":"The diagnosis of idiopathic pulmonary fibrosis: Current and future approaches","volume":"5","author":"Martinez","year":"2017","journal-title":"Lancet Respir. Med."},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"370","DOI":"10.1016\/j.amjms.2019.02.006","article-title":"Current and Future Idiopathic Pulmonary Fibrosis Therapy","volume":"357","author":"Richeldi","year":"2019","journal-title":"Am. J. Med. Sci."},{"key":"ref_128","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1016\/j.ebiom.2019.10.050","article-title":"Endotype\u2013phenotyping may predict a treatment response in progressive fibrosing interstitial lung disease","volume":"50","author":"Weigt","year":"2019","journal-title":"EBioMedicine"},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"239","DOI":"10.7150\/ijbs.29063","article-title":"Clopidogrel Reduces Fibronectin Accumulation and Improves Diabetes-Induced Renal Fibrosis","volume":"15","author":"Zheng","year":"2019","journal-title":"Int. J. Biol. Sci."},{"key":"ref_130","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1007\/s10557-013-6471-z","article-title":"Inhibition of Platelet Activation by Clopidogrel Prevents Hypertension-Induced Cardiac Inflammation and Fibrosis","volume":"27","author":"Jia","year":"2013","journal-title":"Cardiovasc. Drugs Ther."},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"PA596","DOI":"10.1183\/13993003.congress-2018.PA596","article-title":"Phase 2B dose selection of BG00011 for the treatment of idiopathic pulmonary fibrosis (IPF)","volume":"52","author":"Arefayene","year":"2018","journal-title":"Mech. Lung Inj. Repair"},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"723","DOI":"10.1083\/jcb.200312172","article-title":"Integrin alphaVbeta6-mediated activation of latent TGF-beta requires the latent TGF-beta binding protein-1","volume":"165","author":"Annes","year":"2004","journal-title":"J. Cell Biol."},{"key":"ref_133","doi-asserted-by":"crossref","first-page":"3100","DOI":"10.1111\/bph.14362","article-title":"Platelets, autotaxin and lysophosphatidic acid signalling: Win-win factors for cancer metastasis","volume":"175","author":"Leblanc","year":"2018","journal-title":"Br. J. Pharmacol."},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1016\/S2213-2600(19)30172-9","article-title":"Long-term treatment with recombinant human pentraxin 2 protein in patients with idiopathic pulmonary fibrosis: An open-label extension study","volume":"7","author":"Raghu","year":"2019","journal-title":"Lancet Respir. Med."},{"key":"ref_135","doi-asserted-by":"crossref","first-page":"e87446","DOI":"10.1172\/jci.insight.87446","article-title":"Pentraxin-2 suppresses c-Jun\/AP-1 signaling to inhibit progressive fibrotic disease","volume":"1","author":"Nakagawa","year":"2016","journal-title":"JCI Insight"},{"key":"ref_136","doi-asserted-by":"crossref","first-page":"2630","DOI":"10.1016\/S0140-6736(16)00232-4","article-title":"Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): A phase 2, randomised, controlled trial","volume":"387","author":"Khanna","year":"2016","journal-title":"Lancet"},{"key":"ref_137","doi-asserted-by":"crossref","first-page":"2518","DOI":"10.1056\/NEJMoa1903076","article-title":"Nintedanib for Systemic Sclerosis\u2013Associated Interstitial Lung Disease","volume":"380","author":"Distler","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_138","doi-asserted-by":"crossref","first-page":"1941","DOI":"10.1136\/annrheumdis-2016-210823","article-title":"Nintedanib inhibits macrophage activation and ameliorates vascular and fibrotic manifestations in the Fra2 mouse model of systemic sclerosis","volume":"76","author":"Huang","year":"2017","journal-title":"Ann. Rheum. Dis."},{"key":"ref_139","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1189\/jlb.0409236","article-title":"The phenotype of murine wound macrophages","volume":"87","author":"Daley","year":"2009","journal-title":"J. Leukoc. Biol."},{"key":"ref_140","doi-asserted-by":"crossref","unstructured":"Rendin, L.E., L\u00f6fdahl, A., \u00c5hrman, E., M\u00fcller, C., Notermans, T., Michalikov\u00e1, B., Rosmark, O., Zhou, X.-H., Dellgren, G., and Silverborn, M. (2019). Matrisome Properties of Scaffolds Direct Fibroblasts in Idiopathic Pulmonary Fibrosis. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20164013"},{"key":"ref_141","doi-asserted-by":"crossref","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","article-title":"Nintedanib in Progressive Fibrosing Interstitial Lung Diseases","volume":"381","author":"Flaherty","year":"2019","journal-title":"N. Engl. J. Med."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/22\/1\/225\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:47:04Z","timestamp":1760179624000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/22\/1\/225"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12,28]]},"references-count":141,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,1]]}},"alternative-id":["ijms22010225"],"URL":"https:\/\/doi.org\/10.3390\/ijms22010225","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,12,28]]}}}