Pathogenesis of systemic sclerosis: recent insights of molecular and cellular mechanisms and therapeutic opportunities

TLR4 enhances TGF-β signaling and hepatic fibrosis

Seki, E., De Minicis, S., Österreicher, C. H., Kluwe, J., Osawa, Y., Brenner, D. A., & Schwabe, R. F. (2007) · Nature Medicine · doi:10.1038/nm1663 · Cited by 1,942

SPECIFICITY AND VERSATILITY IN TGF-β SIGNALING THROUGH SMADS

Feng, X.-H., & Derynck, R. (2005) · Annual Review of Cell and Developmental Biology · doi:10.1146/annurev.cellbio.21.022404.142018 · Cited by 1,869

Fibrotic disease and the TH1/TH2 paradigm

Wynn, T. A. (2004) · Nature Reviews Immunology · doi:10.1038/nri1412 · Cited by 1,765

Formation and function of the myofibroblast during tissue repair

Hinz, B. (2007) · Journal of Investigative Dermatology · doi:10.1038/sj.jid.5700613 · Cited by 1,658

The emerging roles of YAP and TAZ in cancer

Moroishi, T., Hansen, C. G., & Guan, K.-L. (2015) · Nature Reviews Cancer · doi:10.1038/nrc3876 · Cited by 1,145

Transduction of mechanical and cytoskeletal cues by YAP and TAZ

Halder, G., Dupont, S., & Piccolo, S. (2012) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm3416 · Cited by 985

Peripheral Blood Fibrocytes: Differentiation Pathway and Migration to Wound Sites

Abe, R., Donnelly, S. C., Peng, T., Bucala, R., & Metz, C. N. (2001) · The Journal of Immunology · doi:10.4049/jimmunol.166.12.7556 · Cited by 967 · cited 1×

The lysophosphatidic acid receptor LPA1 links pulmonary fibrosis to lung injury by mediating fibroblast recruitment and vascular leak

Tager, A. M., LaCamera, P., Shea, B. S., Campanella, G. S., Selman, M., Zhao, Z., Polosukhin, V., Wain, J., Karimi-Shah, B. A., Kim, N. D., et al. (2008) · Nature Medicine · doi:10.1038/nm1685 · Cited by 823

Digital ulcers in systemic sclerosis: Prevention by treatment with bosentan, an oral endothelin receptor antagonist

Korn, J. H., Mayes, M., Matucci Cerinic, M., Rainisio, M., Pope, J., Hachulla, E., Rich, E., Carpentier, P., Molitor, J., Seibold, J. R., et al. (2004) · Arthritis & Rheumatism · doi:10.1002/art.20676 · Cited by 728

The myofibroblast: Paradigm for a mechanically active cell

Hinz, B. (2010) · Journal of Biomechanics · doi:10.1016/j.jbiomech.2009.09.020 · Cited by 698

Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faSScinate): a phase 2, randomised, controlled trial

Khanna, D., Denton, C. P., Jahreis, A., van Laar, J. M., Frech, T. M., Anderson, M. E., Baron, M., Chung, L., Fierlbeck, G., Lakshminarayanan, S., et al. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00232-4 · Cited by 683

The Hippo pathway effectors TAZ and YAP in development, homeostasis and disease

Varelas, X. (2014) · Development · doi:10.1242/dev.102376 · Cited by 665

An Updated Overview on Wnt Signaling Pathways: A Prelude for More

Rao, T. P., & Kühl, M. (2010) · Circulation Research · doi:10.1161/circresaha.110.219840 · Cited by 638

Autoantibodies and microvascular damage are independent predictive factors for the progression of Raynaud's phenomenon to systemic sclerosis: A twenty‐year prospective study of 586 patients, with validation of proposed criteria for early systemic sclerosis

Koenig, M., Joyal, F., Fritzler, M. J., Roussin, A., Abrahamowicz, M., Boire, G., Goulet, J., Rich, É., Grodzicky, T., Raymond, Y., et al. (2008) · Arthritis & Rheumatism · doi:10.1002/art.24038 · Cited by 615

Stimulatory Autoantibodies to the PDGF Receptor in Systemic Sclerosis

Svegliati Baroni, S., Santillo, M., Bevilacqua, F., Luchetti, M., Spadoni, T., Mancini, M., Fraticelli, P., Sambo, P., Funaro, A., Kazlauskas, A., et al. (2006) · New England Journal of Medicine · doi:10.1056/nejmoa052955 · Cited by 585

Endothelial-to-Mesenchymal Transition in Pulmonary Hypertension

Ranchoux, B., Antigny, F., Rucker-Martin, C., Hautefort, A., Péchoux, C., Bogaard, H. J., Dorfmüller, P., Remy, S., Lecerf, F., Planté, S., et al. (2015) · Circulation · doi:10.1161/circulationaha.114.008750 · Cited by 576

Imatinib mesylate inhibits the profibrogenic activity of TGF-β and prevents bleomycin-mediated lung fibrosis

Daniels, C. E., Wilkes, M. C., Edens, M., Kottom, T. J., Murphy, S. J., LIMPER, A. H., & Leof, E. B. (2004) · Journal of Clinical Investigation · doi:10.1172/jci19603 · Cited by 574

Bosentan treatment of digital ulcers related to systemic sclerosis: results from the RAPIDS-2 randomised, double-blind, placebo-controlled trial

Matucci-Cerinic, M., Denton, C. P., Furst, D. E., Mayes, M. D., Hsu, V. M., Carpentier, P., Wigley, F. M., Black, C. M., Fessler, B. J., Merkel, P. A., et al. (2011) · Annals of the Rheumatic Diseases · doi:10.1136/ard.2010.130658 · Cited by 498

Role of TAZ as Mediator of Wnt Signaling

Azzolin, L., Zanconato, F., Bresolin, S., Forcato, M., Basso, G., Bicciato, S., Cordenonsi, M., & Piccolo, S. (2012) · Cell · doi:10.1016/j.cell.2012.11.027 · Cited by 493

IL-1R1/MyD88 signaling and the inflammasome are essential in pulmonary inflammation and fibrosis in mice

Gasse, P., Mary, C., Guenon, I., Noulin, N., Charron, S., Schnyder-Candrian, S., Schnyder, B., Akira, S., Quesniaux, V. F. J., Lagente, V., et al. (2007) · Journal of Clinical Investigation · doi:10.1172/jci32285 · Cited by 481

Review: Evidence That Systemic Sclerosis Is a Vascular Disease

Matucci‐Cerinic, M., Kahaleh, B., & Wigley, F. M. (2013) · Arthritis & Rheumatism · doi:10.1002/art.37988 · Cited by 452

Angiotensin II and Vascular Injury

Montezano, A. C., Nguyen Dinh Cat, A., Rios, F. J., & Touyz, R. M. (2014) · Current Hypertension Reports · doi:10.1007/s11906-014-0431-2 · Cited by 402

β-catenin activation is necessary and sufficient to specify the dorsal dermal fate in the mouse

Atit, R., Sgaier, S. K., Mohamed, O. A., Taketo, M. M., Dufort, D., Joyner, A. L., Niswander, L., & Conlon, R. A. (2006) · Developmental Biology · doi:10.1016/j.ydbio.2006.04.449 · Cited by 400 · cited 1×

Sequential dermal microvascular and perivascular changes in the development of scleroderma

Prescott, R. J., Freemont, A. J., Jones, C. J. P., Hoyland, J., & Fielding, P. (1992) · The Journal of Pathology · doi:10.1002/path.1711660307 · Cited by 391

Imatinib mesylate reduces production of extracellular matrix and prevents development of experimental dermal fibrosis

Distler, J. H. W., Jüngel, A., Huber, L. C., Schulze‐Horsel, U., Zwerina, J., Gay, R. E., Michel, B. A., Hauser, T., Schett, G., Gay, S., et al. (2007) · Arthritis & Rheumatism · doi:10.1002/art.22314 · Cited by 383

Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma.

Sgonc, R., Gruschwitz, M. S., Dietrich, H., Recheis, H., Gershwin, M. E., & Wick, G. (1996) · Journal of Clinical Investigation · doi:10.1172/jci118851 · Cited by 374

Endothelial to mesenchymal transition contributes to endothelial dysfunction in pulmonary arterial hypertension

Good, R. B., Gilbane, A. J., Trinder, S. L., Denton, C. P., Coghlan, G., Abraham, D. J., & Holmes, A. M. (2015) · The American Journal of Pathology · doi:10.1016/j.ajpath.2015.03.019 · Cited by 349

Association between enhanced type I collagen expression and epigenetic repression of the FLI1 gene in scleroderma fibroblasts

Wang, Y., Fan, P., & Kahaleh, B. (2006) · Arthritis & Rheumatism · doi:10.1002/art.21948 · Cited by 347

Myofibroblasts in murine cutaneous fibrosis originate from adiponectin-positive intradermal progenitors

Marangoni, R. G., Korman, B. D., Wei, J., Wood, T. A., Graham, L. V., Whitfield, M. L., Scherer, P. E., Tourtellotte, W. G., & Varga, J. (2015) · Arthritis & Rheumatology · doi:10.1002/art.38990 · Cited by 337

Transforming growth factor-β2 promotes Snail-mediated endothelial–mesenchymal transition through convergence of Smad-dependent and Smad-independent signalling

Medici, D., Potenta, S., & Kalluri, R. (2011) · Biochemical Journal · doi:10.1042/bj20101500 · Cited by 316

Fibrosis—a lethal component of systemic sclerosis

Ho, Y. Y., Lagares, D., Tager, A. M., & Kapoor, M. (2014) · Nature Reviews Rheumatology · doi:10.1038/nrrheum.2014.53 · Cited by 306

Experimental myocardial infarction triggers canonical Wnt signaling and endothelial-to-mesenchymal transition

Aisagbonhi, O., Rai, M., Ryzhov, S., Atria, N., Feoktistov, I., & Hatzopoulos, A. K. (2011) · Disease Models & Mechanisms · doi:10.1242/dmm.006510 · Cited by 302

Involvement of functional autoantibodies against vascular receptors in systemic sclerosis

Riemekasten, G., Philippe, A., Näther, M., Slowinski, T., Müller, D. N., Heidecke, H., Matucci-Cerinic, M., Czirják, L., Lukitsch, I., Becker, M., et al. (2011) · Annals of the Rheumatic Diseases · doi:10.1136/ard.2010.135772 · Cited by 302

Transforming growth factor β as a therapeutic target in systemic sclerosis

Varga, J., & Pasche, B. (2009) · Nature Reviews Rheumatology · doi:10.1038/nrrheum.2009.26 · Cited by 295

The Angiotensin-Converting Enzyme 2/Angiogenesis-(1–7)/Mas Axis Confers Cardiopulmonary Protection against Lung Fibrosis and Pulmonary Hypertension

Shenoy, V., Ferreira, A. J., Qi, Y., Fraga-Silva, R. A., Díez-Freire, C., Dooies, A., Jun, J. Y., Sriramula, S., Mariappan, N., Pourang, D., et al. (2010) · American Journal of Respiratory and Critical Care Medicine · doi:10.1164/rccm.200912-1840oc · Cited by 281

Endothelial to Mesenchymal Transition (EndoMT) in the Pathogenesis of Human Fibrotic Diseases

Piera-Velazquez, S., Mendoza, F., & Jimenez, S. (2016) · Journal of Clinical Medicine · doi:10.3390/jcm5040045 · Cited by 273

Increased PDGFRα Activation Disrupts Connective Tissue Development and Drives Systemic Fibrosis

Olson, L. E., & Soriano, P. (2009) · Developmental Cell · doi:10.1016/j.devcel.2008.12.003 · Cited by 264

WNT Signaling in Lung Disease: A Failure or a Regeneration Signal?

Königshoff, M., & Eickelberg, O. (2010) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2008-0485tr · Cited by 258

Inflammatory Cytokines Promote Mesenchymal Transformation in Embryonic and Adult Valve Endothelial Cells

Mahler, G. J., Farrar, E. J., & Butcher, J. T. (2013) · Arteriosclerosis, Thrombosis, and Vascular Biology · doi:10.1161/atvbaha.112.300504 · Cited by 236

Wnt/β‐catenin signaling is hyperactivated in systemic sclerosis and induces Smad‐dependent fibrotic responses in mesenchymal cells

Wei, J., Fang, F., Lam, A. P., Sargent, J. L., Hamburg, E., Hinchcliff, M. E., Gottardi, C. J., Atit, R., Whitfield, M. L., & Varga, J. (2012) · Arthritis & Rheumatism · doi:10.1002/art.34424 · Cited by 226

IL-1β and TGFβ2 synergistically induce endothelial to mesenchymal transition in an NFκB-dependent manner

Maleszewska, M., Moonen, J.-R. A. J., Huijkman, N., van de Sluis, B., Krenning, G., & Harmsen, M. C. (2013) · Immunobiology · doi:10.1016/j.imbio.2012.05.026 · Cited by 220

Lysophosphatidic Acid Induces αvβ6 Integrin-Mediated TGF-β Activation via the LPA2 Receptor and the Small G Protein Gαq

Xu, M. Y., Porte, J., Knox, A. J., Weinreb, P. H., Maher, T. M., Violette, S. M., McAnulty, R. J., Sheppard, D., & Jenkins, G. (2009) · The American Journal of Pathology · doi:10.2353/ajpath.2009.080160 · Cited by 217

Cellular and molecular aspects of vascular dysfunction in systemic sclerosis

Trojanowska, M. (2010) · Nature Reviews Rheumatology · doi:10.1038/nrrheum.2010.102 · Cited by 217

Canonical Wnt signaling induces skin fibrosis and subcutaneous lipoatrophy: a novel mouse model for scleroderma?

Wei, J., Melichian, D., Komura, K., Hinchcliff, M., Lam, A. P., Lafyatis, R., Gottardi, C. J., MacDougald, O. A., & Varga, J. (2011) · Arthritis & Rheumatism · doi:10.1002/art.30312 · Cited by 214

Increased levels of endothelin-1 and differential endothelin type A and B receptor expression in scleroderma-associated fibrotic lung disease.

Abraham, D., Vancheeswaran, R., Dashwood, M. R., Rajkumar, V. S., Pantelides, P., Xu, S. W., du Bois, R. M., & Black, C. M. (1997) · Europe PMC (PubMed Central) · Cited by 211

Selective expression of connective tissue growth factor in fibroblasts in vivo promotes systemic tissue fibrosis

Sonnylal, S., Shi‐Wen, X., Leoni, P., Naff, K., Van Pelt, C. S., Nakamura, H., Leask, A., Abraham, D., Bou‐Gharios, G., & de Crombrugghe, B. (2010) · Arthritis & Rheumatism · doi:10.1002/art.27382 · Cited by 196

Endothelial Fli1 Deficiency Impairs Vascular Homeostasis

Asano, Y., Stawski, L., Hant, F., Highland, K., Silver, R., Szalai, G., Watson, D. K., & Trojanowska, M. (2010) · The American Journal of Pathology · doi:10.2353/ajpath.2010.090593 · Cited by 194

Prostaglandin F2α receptor signaling facilitates bleomycin-induced pulmonary fibrosis independently of transforming growth factor-β

Oga, T., Matsuoka, T., Yao, C., Nonomura, K., Kitaoka, S., Sakata, D., Kita, Y., Tanizawa, K., Taguchi, Y., Chin, K., et al. (2009) · Nature Medicine · doi:10.1038/nm.2066 · Cited by 190

Capillary Regeneration in Scleroderma: Stem Cell Therapy Reverses Phenotype?

Fleming, J. N., Nash, R. A., McLeod, D. O., Fiorentino, D. F., Shulman, H. M., Connolly, M. K., Molitor, J. A., Henstorf, G., Lafyatis, R., Pritchard, D. K., et al. (2008) · PLoS ONE · doi:10.1371/journal.pone.0001452 · Cited by 188

Vasculopathy in scleroderma

Asano, Y., & Sato, S. (2015) · Seminars in Immunopathology · doi:10.1007/s00281-015-0505-5 · Cited by 187

Selective upregulation of platelet-derived growth factor alpha receptors by transforming growth factor beta in scleroderma fibroblasts

Yamakage, A., Kikuchi, K., Smith, E. A., LeRoy, E. C., & Trojanowska, M. (1992) · The Journal of experimental medicine · doi:10.1084/jem.175.5.1227 · Cited by 184

Angiotensin-Converting Enzyme 2/Angiotensin-(1-7)/Mas Axis Protects against Lung Fibrosis by Inhibiting the MAPK/NF-κB Pathway

Meng, Y., Yu, C.-H., Li, W., Li, T., Luo, W., Huang, S., Wu, P.-S., Cai, S.-X., & Li, X. (2014) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2012-0451oc · Cited by 181

Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension

Tamby, M. C. (2005) · Thorax · doi:10.1136/thx.2004.029082 · Cited by 174

Increased frequency of circulating Th22 in addition to Th17 and Th2 lymphocytes in systemic sclerosis: association with interstitial lung disease

Truchetet, M.-E., Brembilla, N. C., Montanari, E., Allanore, Y., & Chizzolini, C. (2011) · Arthritis Research & Therapy · doi:10.1186/ar3486 · Cited by 173

Amelioration of dermal fibrosis by genetic deletion or pharmacologic antagonism of lysophosphatidic acid receptor 1 in a mouse model of scleroderma

Castelino, F. V., Seiders, J., Bain, G., Brooks, S. F., King, C. D., Swaney, J. S., Lorrain, D. S., Chun, J., Luster, A. D., & Tager, A. M. (2011) · Arthritis & Rheumatism · doi:10.1002/art.30262 · Cited by 168

Induction of interferon‐α by scleroderma sera containing autoantibodies to topoisomerase I: Association of higher interferon‐α activity with lung fibrosis

Kim, D., Peck, A., Santer, D., Patole, P., Schwartz, S. M., Molitor, J. A., Arnett, F. C., & Elkon, K. B. (2008) · Arthritis & Rheumatism · doi:10.1002/art.23486 · Cited by 166

Autoantibodies to angiotensin and endothelin receptors in systemic sclerosis induce cellular and systemic events associated with disease pathogenesis

Kill, A., Tabeling, C., Undeutsch, R., Kühl, A. A., Günther, J., Radic, M., Becker, M. O., Heidecke, H., Worm, M., Witzenrath, M., et al. (2014) · Arthritis Research & Therapy · doi:10.1186/ar4457 · Cited by 166

Hedgehog signaling controls fibroblast activation and tissue fibrosis in systemic sclerosis

Horn, A., Palumbo, K., Cordazzo, C., Dees, C., Akhmetshina, A., Tomcik, M., Zerr, P., Avouac, J., Gusinde, J., Zwerina, J., et al. (2012) · Arthritis & Rheumatism · doi:10.1002/art.34444 · Cited by 160

CCN2 is required for bleomycin‐induced skin fibrosis in mice

Liu, S., Shiwen, X., Abraham, D., & Leask, A. (2010) · Arthritis & Rheumatism · doi:10.1002/art.30074 · Cited by 157

C3a and C5a receptor antagonists ameliorate endothelial-myofibroblast transition via the Wnt/β-catenin signaling pathway in diabetic kidney disease

Li, L., Chen, L., Zang, J., Tang, X., Liu, Y., Zhang, J., Bai, L., Yin, Q., Lu, Y., Cheng, J., et al. (2015) · Metabolism · doi:10.1016/j.metabol.2015.01.014 · Cited by 157

JAK‐2 as a novel mediator of the profibrotic effects of transforming growth factor β in systemic sclerosis

Dees, C., Tomčík, M., Palumbo‐Zerr, K., Distler, A., Beyer, C., Lang, V. R., Horn, A., Zerr, P., Zwerina, J., Gelse, K., et al. (2012) · Arthritis & Rheumatism · doi:10.1002/art.34500 · Cited by 157

Nuclear β-Catenin Is Increased in Systemic Sclerosis Pulmonary Fibrosis and Promotes Lung Fibroblast Migration and Proliferation

Lam, A. P., Flozak, A. S., Russell, S., Wei, J., Jain, M., Mutlu, G. M., Budinger, G. R. S., Feghali‐Bostwick, C. A., Varga, J., & Gottardi, C. J. (2011) · American Journal of Respiratory Cell and Molecular Biology · doi:10.1165/rcmb.2010-0113oc · Cited by 156

Sirt1 regulates canonical TGF-β signalling to control fibroblast activation and tissue fibrosis

Zerr, P., Palumbo‐Zerr, K., Huang, J., Tomčík, M., Šumová, B., Distler, O., Schett, G. A., & Distler, J. H. W. (2014) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2014-205740 · Cited by 153

Integrating developmental signals: a Hippo in the (path)way

Mauviel, A., Nallet-Staub, F., & Varelas, X. (2011) · Oncogene · doi:10.1038/onc.2011.363 · Cited by 141

Epithelial Cells Promote Fibroblast Activation via IL-1α in Systemic Sclerosis

Aden, N., Nuttall, A., Shiwen, X., de Winter, P., Leask, A., Black, C. M., Denton, C. P., Abraham, D., & Stratton, R. J. (2010) · Journal of Investigative Dermatology · doi:10.1038/jid.2010.120 · Cited by 138

Matrix metalloproteinase 12–dependent cleavage of urokinase receptor in systemic sclerosis microvascular endothelial cells results in impaired angiogenesis

D’Alessio, S., Fibbi, G., Cinelli, M., Guiducci, S., Rosso, A. D., Margheri, F., Serratì, S., Pucci, M., Kahaleh, B., Fan, P., et al. (2004) · Arthritis & Rheumatism · doi:10.1002/art.20562 · Cited by 137

Blockade of Interleukin-6 Receptor Alleviates Disease in Mouse Model of Scleroderma

Kitaba, S., Murota, H., Terao, M., Azukizawa, H., Terabe, F., Shima, Y., Fujimoto, M., Tanaka, T., Naka, T., Kishimoto, T., et al. (2011) · American Journal Of Pathology · doi:10.1016/j.ajpath.2011.09.013 · Cited by 131

Peroxisome proliferator‐activated receptor‐γ abrogates Smad‐dependent collagen stimulation by targeting the p300 transcriptional coactivator

Ghosh, A. K., Bhattacharyya, S., Wei, J., Kim, S., Barak, Y., Mori, Y., & Varga, J. (2009) · The FASEB Journal · doi:10.1096/fj.08-128736 · Cited by 129

Effector CD8+ T cells in systemic sclerosis patients produce abnormally high levels of interleukin‐13 associated with increased skin fibrosis

Fuschiotti, P., Medsger, T. A., & Morel, P. A. (2009) · Arthritis & Rheumatism · doi:10.1002/art.24432 · Cited by 129

Vascular Disease in Scleroderma

Wigley, F. M. (2008) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-008-8106-x · Cited by 126

Transcription Factor Fos-Related Antigen-2 Induces Progressive Peripheral Vasculopathy in Mice Closely Resembling Human Systemic Sclerosis

Maurer, B., Busch, N., Jüngel, A., Pileckytė, M., Gay, R. E., Michel, B. A., Schett, G. A., Gay, S., Distler, J. H. W., & Distler, O. (2009) · Circulation · doi:10.1161/circulationaha.109.855114 · Cited by 126

Endothelial Cells Expressing Endothelial and Mesenchymal Cell Gene Products in Lung Tissue From Patients With Systemic Sclerosis–Associated Interstitial Lung Disease

Mendoza, F. A., Piera‐Velazquez, S., Farber, J. L., Feghali‐Bostwick, C., & Jiménez, S. A. (2016) · Arthritis & Rheumatology · doi:10.1002/art.39421 · Cited by 121

Angiotensin II in the lesional skin of systemic sclerosis patients contributes to tissue fibrosis via angiotensin II type 1 receptors

Kawaguchi, Y., Takagi, K., Hara, M., Fukasawa, C., Sugiura, T., Nishimagi, E., Harigai, M., & Kamatani, N. (2004) · Arthritis & Rheumatism · doi:10.1002/art.11364 · Cited by 120

Inflammatory cytokines induce the transformation of human dermal microvascular endothelial cells into myofibroblasts: a potential role in skin fibrogenesis

Chaudhuri, V., Zhou, L. Y., & Karasek, M. A. (2006) · Journal of Cutaneous Pathology · doi:10.1111/j.1600-0560.2006.00584.x · Cited by 116

Increased expression of platelet‐derived growth factor type b receptors in the skin of patients with systemic sclerosis

Klareskog, L., Gustafsson, R., Scheynius, A., & Hällgren, R. (1990) · Arthritis & Rheumatism · doi:10.1002/art.1780331011 · Cited by 115

Lipopolysaccharide induces a fibrotic‐like phenotype in endothelial cells

Echeverría, C., Montorfano, I., Sarmiento, D., Becerra, Á., Nuñez‐Villena, F., Figueroa, X. F., Cabello‐Verrugio, C., Elorza, Á. A., Riedel, C. A., & Simón, F. (2013) · Journal of Cellular and Molecular Medicine · doi:10.1111/jcmm.12066 · Cited by 115

Targeting ADAM‐17/notch signaling abrogates the development of systemic sclerosis in a murine model

Kavian, N., Servettaz, A., Mongaret, C., Wang, A. A., Nicco, C., Chéreau, C., Grange, P., Vuiblet, V., Birembaut, P. L., Diébold, M.-D., et al. (2010) · Arthritis & Rheumatism · doi:10.1002/art.27626 · Cited by 113

Function Blocking Autoantibodies Against Matrix Metalloproteinase-1 in Patients with Systemic Sclerosis

Sato, S., Hayakawa, I., Hasegawa, M., Takehara, K., & Fujimoto, M. (2003) · Journal of Investigative Dermatology · doi:10.1046/j.1523-1747.2003.12097.x · Cited by 112

Diminished Induction of Skin Fibrosis in Mice with MCP-1 Deficiency

Ferreira, A. M., Takagawa, S., Fresco, R., Zhu, X., Varga, J., & DiPietro, L. A. (2006) · Journal of Investigative Dermatology · doi:10.1038/sj.jid.5700302 · Cited by 112

Stimulation of Transforming Growth Factor-β1-Induced Endothelial-To-Mesenchymal Transition and Tissue Fibrosis by Endothelin-1 (ET-1): A Novel Profibrotic Effect of ET-1

Wermuth, P. J., Li, Z., Mendoza, F. A., & Jiménez, S. A. (2016) · PLoS ONE · doi:10.1371/journal.pone.0161988 · Cited by 110

SIRT3 is attenuated in systemic sclerosis skin and lungs, and its pharmacologic activation mitigates organ fibrosis

Akamata, K., Wei, J., Bhattacharyya, M., Cheresh, P., Bonner, M. Y., Arbiser, J. L., Raparia, K., Gupta, M. P., Kamp, D. W., & Varga, J. (2016) · Oncotarget · doi:10.18632/oncotarget.12504 · Cited by 109

Compromised peroxisomes in idiopathic pulmonary fibrosis, a vicious cycle inducing a higher fibrotic response via TGF-β signaling

Oruqaj, G., Karnati, S., Vijayan, V., Kotarkonda, L. K., Boateng, E., Zhang, W., Ruppert, C., Günther, A., Shi, W., & Baumgart‐Vogt, E. (2015) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1415111112 · Cited by 108

Protein kinase Cδ and c‐Abl kinase are required for transforming growth factor β induction of endothelial–mesenchymal transition in vitro

Li, Z., & Jiménez, S. A. (2011) · Arthritis & Rheumatism · doi:10.1002/art.30317 · Cited by 99

Inactivation of urokinase-type plasminogen activator receptor (uPAR) gene induces dermal and pulmonary fibrosis and peripheral microvasculopathy in mice: a new model of experimental scleroderma?

Manetti, M., Rosa, I., Milia, A. F., Guiducci, S., Carmeliet, P., Ibba‐Manneschi, L., & Matucci‐Cerinic, M. (2013) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2013-203706 · Cited by 97

The Histone Deacetylase Sirtuin 1 Is Reduced in Systemic Sclerosis and Abrogates Fibrotic Responses by Targeting Transforming Growth Factor β Signaling

Wei, J., Ghosh, A. K., Chu, H., Fang, F., Hinchcliff, M., Wang, J., Marangoni, R. G., & Varga, J. (2015) · Arthritis & Rheumatology · doi:10.1002/art.39061 · Cited by 96

Scleroderma Peripheral B Lymphocytes Secrete Interleukin‐6 and Transforming Growth Factor β and Activate Fibroblasts

Dumoitier, N., Chaigne, B., Régent, A., Lofek, S., Mhibik, M., Dorfmüller, P., Terrier, B., London, J., BEREZNE, A., Tamas, N., et al. (2016) · Arthritis & Rheumatology · doi:10.1002/art.40016 · Cited by 96

Interferon‐γ reverses the stimulation of collagen but not fibronectin gene expression by transforming growth factor‐ β in normal human fibroblasts

Varga, J., Olsen, A. S., HERHAL, J., Constantine, G. M., Rosenbloom, J., & Jiménez, S. A. (1990) · European Journal of Clinical Investigation · doi:10.1111/j.1365-2362.1990.tb01890.x · Cited by 95

Interferon‐γ promotes vascular remodeling in human microvascular endothelial cells by upregulating endothelin (ET)‐1 and transforming growth factor (TGF) β2

Chrobak, I., Lenna, S., Stawski, L., & Trojanowska, M. E. (2013) · Journal of Cellular Physiology · doi:10.1002/jcp.24337 · Cited by 93

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Taniguchi, T., Asano, Y., Akamata, K., Noda, S., Takahashi, T., Ichimura, Y., Toyama, T., Trojanowska, M., & Sato, S. (2014) · Arthritis & Rheumatology · doi:10.1002/art.38948 · Cited by 92

Autoantibody against matrix metalloproteinase-3 in patients with systemic sclerosis

Nishijima, C., Hayakawa, I., Matsushita, T., Komura, K., Hasegawa, M., Takehara, K., & Sato, S. (2004) · Clinical & Experimental Immunology · doi:10.1111/j.1365-2249.2004.02615.x · Cited by 92

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Rodríguez‐Pascual, F., Busnadiego, Ó., & González, J. (2014) · Life Sciences · doi:10.1016/j.lfs.2013.12.024 · Cited by 90

Epigenetic repression of bone morphogenetic protein receptor II expression in scleroderma

Wang, Y., & Kahaleh, B. (2013) · Journal of Cellular and Molecular Medicine · doi:10.1111/jcmm.12105 · Cited by 86

Prolonged β-catenin stabilization and tcf-dependent transcriptional activation in hyperplastic cutaneous wounds

Cheon, S. S., Poon, R. W. M., Yu, C., Khoury, M., Shenker, R., Fish, J. S., & Alman, B. A. (2005) · Laboratory Investigation · doi:10.1038/labinvest.3700237 · Cited by 86

Lysophosphatidic acid-1-receptor targeting agents for fibrosis

Rancoule, C., Pradère, J., Gonzalez, J., Klein, J., Valet, P., Bascands, J., Schanstra, J. P., & Saulnier‐Blache, J. S. (2011) · Expert Opinion on Investigational Drugs · doi:10.1517/13543784.2011.566864 · Cited by 86

An Overview of Hedgehog Signaling in Fibrosis

Hu, L., Lin, X., Lu, H., Chen, B., & Bai, Y. (2014) · Molecular Pharmacology · doi:10.1124/mol.114.095141 · Cited by 86

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Bhattacharyya, S., Ishida, W., Wu, M., Wilkes, M. C., Mori, Y., Hinchcliff, M., Leof, E. B., & Varga, J. (2009) · Oncogene · doi:10.1038/onc.2008.479 · Cited by 85

Epithelial Fli1 deficiency drives systemic autoimmunity and fibrosis: Possible roles in scleroderma

Takahashi, T., Asano, Y., Sugawara, K., Yamashita, T., Nakamura, K., Saigusa, R., Ichimura, Y., Toyama, T., Taniguchi, T., Akamata, K., et al. (2017) · The Journal of Experimental Medicine · doi:10.1084/jem.20160247 · Cited by 85

Studies of the microvascular endothelium in uninvolved skin of patients with systemic sclerosis: direct evidence for a generalized microangiopathy

Freemont, A. J., Hoyland, J. A., Fielding, P., Hodson, N. W., & Jayson, M. I. V. (2008) · British Journal of Dermatology · doi:10.1111/j.1365-2133.1992.tb00100.x · Cited by 84

Endothelin 1 contributes to the effect of transforming growth factor β1 on wound repair and skin fibrosis

Lagares, D., García‐Fernández, R. A., Jiménez, C. L., Magán‐Marchal, N., Busnadiego, O., Lamas, S., & Rodríguez‐Pascual, F. (2010) · Arthritis & Rheumatism · doi:10.1002/art.27307 · Cited by 80

Peroxisome proliferator-activated receptor γ: innate protection from excessive fibrogenesis and potential therapeutic target in systemic sclerosis

Wei, J., Bhattacharyya, S., & Varga, J. (2010) · Current Opinion in Rheumatology · doi:10.1097/bor.0b013e32833de1a7 · Cited by 77

Angiotensin II induces skin fibrosis: a novel mouse model of dermal fibrosis

Stawski, L., Han, R., Bujor, A. M., & Trojanowska, M. (2012) · Arthritis Research & Therapy · doi:10.1186/ar4028 · Cited by 75

Domain 1 of the urokinase‐type plasminogen activator receptor is required for its morphologic and functional, β2 integrin–mediated connection with actin cytoskeleton in human microvascular endothelial cells: Failure of association in systemic sclerosis endothelial cells

Margheri, F., Manetti, M., Serratì, S., Nosi, D., Pucci, M., Matucci‐Cerinic, M., Kahaleh, B., Bazzichi, L. M., Fibbi, G., Ibba‐Manneschi, L., et al. (2006) · Arthritis & Rheumatism · doi:10.1002/art.22263 · Cited by 74

Histone Deacetylase 5 Is Overexpressed in Scleroderma Endothelial Cells and Impairs Angiogenesis via Repression of Proangiogenic Factors

Tsou, P., Wren, J. D., Amin, M. A., Schiopu, E., Fox, D. A., Khanna, D., & Sawalha, A. H. (2016) · Arthritis & Rheumatology · doi:10.1002/art.39828 · Cited by 73

The Fibrotic Phenotype Induced by IGFBP-5 Is Regulated by MAPK Activation and Egr-1-Dependent and -Independent Mechanisms

Yasuoka, H., Hsu, E., Ruiz, X. D., Steinman, R. A., Choi, A. M. K., & Feghali-Bostwick, C. A. (2009) · American Journal Of Pathology · doi:10.2353/ajpath.2009.080991 · Cited by 72

Role of interleukin‐13 in fibrosis, particularly systemic sclerosis

O’Reilly, S. (2013) · BioFactors · doi:10.1002/biof.1117 · Cited by 71

Mechanism of Serum-Mediated Endothelial Injury in Scleroderma: Identification of a Granular Enzyme in Scleroderma Skin and Sera

Kahaleh, M. B., & Fan, P. (1997) · Clinical Immunology and Immunopathology · doi:10.1006/clin.1996.4322 · Cited by 70

Increased Bleomycin-Induced Skin Fibrosis in Mice Lacking the Th1-Specific Transcription Factor T-bet

Lakos, G., Melichian, D. S., Wu, M., & Varga, J. (2006) · Pathobiology · doi:10.1159/000098208 · Cited by 69

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Fang, F., Ooka, K., Sun, X., Shah, R., Bhattacharyya, S., Wei, J., & Varga, J. (2013) · The Journal of Immunology · doi:10.4049/jimmunol.1300376 · Cited by 67

A model of anti-angiogenesis: differential transcriptosome profiling of microvascular endothelial cells from diffuse systemic sclerosis patients

Giusti, B., Fibbi, G., Margheri, F., Serratì, S., Rossi, L., Poggi, F., Lapini, I., Magi, A., Rosso, A. D., Cinelli, M., et al. (2006) · Arthritis Research & Therapy · doi:10.1186/ar2002 · Cited by 62

Caveolin-1 Deficiency Induces Spontaneous Endothelial-to-Mesenchymal Transition in Murine Pulmonary Endothelial Cells in Vitro

Li, Z., Wermuth, P. J., Benn, B. S., Lisanti, M. P., & Jiménez, S. A. (2012) · American Journal Of Pathology · doi:10.1016/j.ajpath.2012.10.022 · Cited by 60

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Briones-Orta, M. A., Tecalco-Cruz, Á. C., Sosa‐Garrocho, M., Caligaris, C., & Macías‐Silva, M. (2011) · Current Molecular Pharmacology · doi:10.2174/1874467211104020141 · Cited by 58

Hedgehog signaling: From basic research to clinical applications

Yao, E., & Chuang, P. (2015) · Journal of the Formosan Medical Association · doi:10.1016/j.jfma.2015.01.005 · Cited by 53

Blockade of PDGF Receptors by Crenolanib Has Therapeutic Effect in Patient Fibroblasts and in Preclinical Models of Systemic Sclerosis

Makino, K., Makino, T., Stawski, L., Mantero, J. C., Lafyatis, R., Simms, R. W., & Trojanowska, M. (2017) · Journal of Investigative Dermatology · doi:10.1016/j.jid.2017.03.032 · Cited by 52

Decreased expression of neuropilin-1 as a novel key factor contributing to peripheral microvasculopathy and defective angiogenesis in systemic sclerosis

Romano, E., Chora, I., Manetti, M., Mazzotta, C., Rosa, I., Bellando-Randone, S., Blagojević, J., Soares, R. A., Avouac, J., Allanore, Y., et al. (2016) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2015-207483 · Cited by 51

MMP-12 Deficiency Attenuates Angiotensin II-Induced Vascular Injury, M2 Macrophage Accumulation, and Skin and Heart Fibrosis

Stawski, L., Haines, P., Fine, A., Rudnicka, L., & Trojanowska, M. E. (2014) · PLoS ONE · doi:10.1371/journal.pone.0109763 · Cited by 51

The c-Abl tyrosine kinase controls protein kinase Cδ-induced Fli-1 phosphorylation in human dermal fibroblasts

Bujor, A. M., Asano, Y., Haines, P., et al. (2011) · Arthritis & Rheumatism · doi:10.1002/art.30284 · Cited by 51

Prevalence of anti-endothelial cell antibodies in idiopathic pulmonary arterial hypertension: Table 1—

Arends, S. J., Damoiseaux, J. G. M. C., Duijvestijn, A. M., Debrus-Palmans, L., Boomars, K. A., Broers, B., Tervaert, J.-W. C., & van Paassen, P. (2010) · European Respiratory Journal · doi:10.1183/09031936.00164209 · Cited by 51

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Saigusa, R., Asano, Y., Taniguchi, T., Yamashita, T., Takahashi, T., Ichimura, Y., Toyama, T., Tamaki, Z., Tada, Y., Sugaya, M., et al. (2014) · Experimental Dermatology · doi:10.1111/exd.12602 · Cited by 46

Glycyrrhizin Ameliorates Fibrosis, Vasculopathy, and Inflammation in Animal Models of Systemic Sclerosis

Yamashita, T., Asano, Y., Taniguchi, T., Nakamura, K., Saigusa, R., Miura, S., Toyama, T., Takahashi, T., Ichimura, Y., Yoshizaki, A., et al. (2016) · Journal of Investigative Dermatology · doi:10.1016/j.jid.2016.08.037 · Cited by 46

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Marut, W., Kavian, N., Servettaz, A., Hua-Huy, T., Nicco, C., Chéreau, C., Weill, B. J., Dinh‐Xuan, A. T., & Batteux, F. (2013) · Arthritis & Rheumatism · doi:10.1002/art.37873 · Cited by 42

Nrf2 exerts cell-autonomous antifibrotic effects: compromised function in systemic sclerosis and therapeutic rescue with a novel heterocyclic chalcone derivative

Wei, J., Zhu, H., Lord, G., Bhattachayya, M., Jones, B., Allaway, G. P., Biswal, S. S., Korman, B. D., Marangoni, R. G., Tourtellotte, W. G., et al. (2016) · Translational research · doi:10.1016/j.trsl.2016.12.002 · Cited by 39

Combined inhibition of morphogen pathways demonstrates additive antifibrotic effects and improved tolerability

Distler, A., Lang, V. R., Vecchio, T. D., Huang, J., Zhang, Y., Beyer, C., Lin, N., Palumbo‐Zerr, K., Distler, O., Schett, G. A., et al. (2014) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2013-204221 · Cited by 38

Macro- and microvascular disease in systemic sclerosis

Kavian, N., & Batteux, F. (2015) · Vascular Pharmacology · doi:10.1016/j.vph.2015.05.015 · Cited by 37

Bosentan and macitentan prevent the endothelial-to-mesenchymal transition (EndoMT) in systemic sclerosis: in vitro study

Corallo, C., Cutolo, M., Kahaleh, B., Pecetti, G., Montella, A., Chirico, C., Soldano, S., Nuti, R., & Giordano, N. G. (2016) · Arthritis Research & Therapy · doi:10.1186/s13075-016-1122-y · Cited by 34

Endothelin Receptor Blockade Ameliorates Vascular Fragility in Endothelial Cell–Specific Fli‐1–Knockout Mice by Increasing Fli‐1 DNA Binding Ability

Akamata, K., Asano, Y., Yamashita, T., Noda, S., Taniguchi, T., Takahashi, T., Ichimura, Y., Toyama, T., Trojanowska, M., & Sato, S. (2015) · Arthritis & Rheumatology · doi:10.1002/art.39062 · Cited by 30

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Wermuth, P. J., Carney, K. R., Mendoza, F. A., Piera-Velázquez, S., & Jiménez, S. A. (2017) · Laboratory Investigation · doi:10.1038/labinvest.2017.23 · Cited by 30

Preventive effect of irbesartan on bleomycin-induced lung injury in mice

Tanaka, J., Tajima, S., Asakawa, K., Sakagami, T., Moriyama, H., Takada, T., Suzuki, E., & Narita, I. (2013) · Respiratory Investigation · doi:10.1016/j.resinv.2012.12.005 · Cited by 29

Capturing the heterogeneity in systemic sclerosis with genome-wide expression profiling

Sargent, J. L., & Whitfield, M. L. (2011) · Expert Review of Clinical Immunology · doi:10.1586/eci.11.41 · Cited by 29

Interferon-Beta, a Decisive Factor in Angiogenesis and Arteriogenesis

Yıldırım, C., Nieuwenhuis, S., Teunissen, P. F. A., et al. (2015) · Journal of Interferon & Cytokine Research · doi:10.1089/jir.2014.0184 · Cited by 27

Activation of the Thromboxane A2 Receptor by 8-Isoprostane Inhibits the Pro-Angiogenic Effect of Vascular Endothelial Growth Factor in Scleroderma

Tsou, P., Amin, M. A., Campbell, P. L., et al. (2015) · Journal of Investigative Dermatology · doi:10.1038/jid.2015.323 · Cited by 26

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Makino, K., Jinnin, M., Aoi, J., Kajihara, I., Makino, T., Fukushima, S., Sakai, K., Nakayama, K., Emoto, N., Yanagisawa, M., et al. (2014) · PLoS ONE · doi:10.1371/journal.pone.0097972 · Cited by 26

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Cabral-Marques, O., & Riemekasten, G. (2017) · Journal of Scleroderma and Related Disorders · doi:10.5301/jsrd.5000241 · Cited by 8

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Skaug, B., Khanna, D., Swindell, W. R., Hinchcliff, M. E., Frech, T. M., Steen, V. D., Hant, F. N., Gordon, J. K., Shah, A. A., Zhu, L., et al. (2020) · Annals of the Rheumatic Diseases · doi:10.1136/annrheumdis-2019-215894 · Cited by 179

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Stawski, L., & Trojanowska, M. (2019) · Connective Tissue Research · doi:10.1080/03008207.2018.1500558 · Cited by 86

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Varjú, C., Kumánovics, G., Czirják, L., Matucci-Cerinic, M., & Minier, T. (2020) · Clinics in Dermatology · doi:10.1016/j.clindermatol.2019.10.010 · Cited by 20