4.2 Review

Systemic Sclerosis Pathogenesis and Emerging Therapies, beyond the Fibroblast

Journal

BIOMED RESEARCH INTERNATIONAL
Volume 2019, Issue -, Pages -

Publisher

HINDAWI LTD
DOI: 10.1155/2019/4569826

Keywords

-

Ask authors/readers for more resources

Systemic sclerosis (SSc) is a complex rheumatologic autoimmune disease in which inflammation, fibrosis, and vasculopathy share several pathogenic pathways that lead to skin and internal organ damage. Recent findings regarding the participation and interaction of the innate and acquired immune system have led to a better understanding of the pathogenesis of the disease and to the identification of new therapeutic targets, many of which have been tested in preclinical and clinical trials with varying results. In this manuscript, we review the state of the art of the pathogenesis of this disease and discuss the main therapeutic targets related to each pathogenic mechanism that have been discovered so far.

Authors

I am an author on this paper
Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.2
Not enough ratings

Secondary Ratings

Novelty
-
Significance
-
Scientific rigor
-
Rate this paper

Recommended

Article Rheumatology

Generation of a Core Set of Items to Develop Classification Criteria for Scleroderma Renal Crisis Using Consensus Methodology

Emily-Ann Butler, Murray Baron, Agnes B. Fogo, Tracy Frech, Cybele Ghossein, Eric Hachulla, Sabrina Hoa, Sindhu R. Johnson, Dinesh Khanna, Luc Mouthon, Mandana Nikpour, Susanna Proudman, Virginia Steen, Edward Stern, John Varga, Christopher Denton, Marie Hudson, April Barnado, Murray Baron, Elana J. Bernstein, Francesco Boin, Yolanda Braun-Moscovici, Flavia V. Castelino, Luis J. Catoggio, Marco Matucci-Cerinic, Lorinda Chung, Philip Clements, Mary Ellen Csuka, Ellen De langhe, Christopher Denton, Joerg Distler, Oliver Distler, Dominique Claire Farge, Aryeh Fischer, Agnes B. Fogo, Tracy Frech, Armando Gabrielli, Cybele Ghossein, Eric Hachulla, Minoru Hasegawa, Samina Hayat, Ariane Herrick, Roger Hesselstrand, Sabrina Hoa, Vivien Hsu, Marie Hudson, Michael Hughes, Nicolas Hunzelmann, Laura Hummers, Florenzo Iannone, Francesca Ingegnoli, Soren Jacobsen, Sindhu R. Johnson, Yasushi Kawaguchi, Dinesh Khanna, Martial Koenig, Masataka Kuwana, Jan Lenaerts, Thierry Martin, Maureen D. Mayes, Zsuzsanna McMahan, Thomas Medsger, Peter Merkel, Sonali Narain, Mandana Nikpour, Voon Ong, John D. Pauling, Janet Pope, Susanna Proudman, Carlos de la Puente, Maureen Rischmueller, Tatiana Sofia Rodriguez-Reyna, Joanne Sahhar, Lesley Ann Saketkoo, Jean-Luc Senecal, Ankoor Shah, Ami A. Shah, Walter Alberto Sifuentes-Giraldo, Richard Silver, Virginia Steen, Edward Stern, Wendy Stevens, Evelyn Sutton, Vivek Thakkar, Gabriele Valentini, Jeska de Vries-Bouwstra, Madelon Vonk, Ulrich A. Walker

ARTHRITIS & RHEUMATOLOGY (2019)

Article Rheumatology

Pan-American League of Associations for Rheumatology (PANLAR) capillaroscopy study group consensus for the format and content of the report in capillaroscopy in rheumatology

Chiara Bertolazzi, Angelica Vargas Guerrero, Tatiana Sofia Rodriguez-Reyna, Hugo Sandoval, Everardo Alvarez-Hernandez, Marcelo Jose Audisio, Eduardo Cabello, Paola Coral-Alvarado, Ericka Diaz, Virginia Duringan, Karinna Espejo, Selma Gallegos, Gabriela Hernandez-Molina, Blanca Herrera, Cristiane Kayser, Maria Eugenia Lara, Genessis Maldonado, Marta N. Mamani, Alejandro Nitsche, Carlos Rios-Acosta, Felix Enrique-Romanini, Maria Sormani de Fonseca, Veronica Silva Vilela, Miguel Angel Villarreal-Alarcon, Marwin Gutierrez

CLINICAL RHEUMATOLOGY (2019)

Article Rheumatology

Changes in skin score in early diffuse cutaneous systemic sclerosis are associated with changes in global disease severity

Boyang Zheng, Tatiana Nevskaya, Carl A. Baxter, Dena R. Ramey, Janet E. Pope, Murray Baron, Marie Hudson, Genevieve Gyger, Maggie J. Larche, Nader A. Khalidi, Ariel Masetto, Evelyn Sutton, David Robinson, Tatiana S. Rodriguez-Reyna, Doug Smith, Carter Thorne, Paul R. Fortin, Marvin J. Fritzler

RHEUMATOLOGY (2020)

Article Psychiatry

Protocol for a partially nested randomised controlled trial to evaluate the effectiveness of the scleroderma patient-centered intervention network COVID-19 home-isolation activities together (SPIN-CHAT) program to reduce anxiety among at-risk scleroderma patients

Brett D. Thombs, Linda Kwakkenbos, Marie-Eve Carrier, Angelica Bourgeault, Lydia Tao, Sami Harb, Maria Gagarine, Danielle Rice, Laura Bustamante, Kelsey Ellis, Delaney Duchek, Yin Wu, Parash Mani Bhandari, Dipika Neupane, Andrea Carboni-Jimenez, Richard S. Henry, Ankur Krishnan, Ying Sun, Brooke Levis, Chen He, Kimberly A. Turner, Andrea Benedetti, Nicole Culos-Reed, Ghassan El-Baalbaki, Shannon Hebblethwaite, Susan J. Bartlett, Laura Dyas, Scott Patten, John Varga, Catherine Fortune, Amy Gietzen, Genevieve Guillot, Nancy Lewis, Karen Nielsen, Michelle Richard, Maureen Sauve, Joep Welling, Murray Baron, Daniel E. Furst, Karen Gottesman, Vanessa Malcarne, Maureen D. Mayes, Luc Mouthon, Warren R. Nielson, Robert Riggs, Fredrick Wigley, Shervin Assassi, Isabelle Boutron, Carolyn Ells, Cornelia van den Ende, Kim Fligelstone, Tracy Frech, Dominique Godard, Daphna Harel, Monique Hinchcliff, Marie Hudson, Sindhu R. Johnson, Maggie Larche, Catarina Leite, Christelle Nguyen, Janet Pope, Alexandra Portales, Francois Rannou, Tatiana Sofia Rodriguez Reyna, Anne A. Schouffoer, Maria E. Suarez-Almazor, Christian Agard, Alexandra Albert, Marc Andre, Guylaine Arsenault, Ilham Benzidia, Elana J. Bernstein, Sabine Berthier, Lyne Bissonnette, Gilles Boire, Alessandra Bruns, Patricia Carreira, Marion Casadevall, Benjamin Chaigne, Lorinda Chung, Pascal Cohen, Chase Correia, Pierre Dagenais, Christopher Denton, Robyn Domsic, Sandrine Dubois, James Dunne, Bertrand Dunogue, Regina Fare, Dominique Farge-Bancel, Paul R. Fortin, Anna Gill, Jessica Gordon, Brigitte Granel-Rey, Genevieve Gyger, Eric Hachulla, Pierre-Yves Hatron, Ariane L. Herrick, Adrian Hij, Sabrina Hoa, Alena Ikic, Niall Jones, Artur Jose de B. Fernandes, Suzanne Kafaja, Nader Khalidi, Marc Lambert, David Launay, Patrick Liang, Helene Maillard, Nancy Maltez, Joanne Manning, Isabelle Marie, Maria Martin, Thierry Martin, Ariel Masetto, Francois Maurier, Arsene Mekinian, Sheila Melchor, Mandana Nikpour, Louis Olagne, Vincent Poindron, Susanna Proudman, Alexis Regent, Sebastien Riviere, David Robinson, Esther Rodriguez, Sophie Roux, Perrine Smets, Doug Smith, Vincent Sobanski, Robert Spiera, Virginia Steen, Wendy Stevens, Evelyn Sutton, Benjamin Terrier, Carter Thorne, Pearce Wilcox, Mara Canedo Ayala, Nora Ostbo

JOURNAL OF PSYCHOSOMATIC RESEARCH (2020)

Article Biochemistry & Molecular Biology

The Transcription Factor SCX is a Potential Serum Biomarker of Fibrotic Diseases

Miguel Ramirez-Aragon, Fernando Hernandez-Sanchez, Tatiana S. Rodriguez-Reyna, Ivette Buendia-Roldan, Gael Guitron-Castillo, Carlos A. Nunez-Alvarez, Diego F. Hernandez-Ramirez, Sergio A. Benavides-Suarez, Alexia Esquinca-Gonzalez, Ana Lilia Torres-Machorro, Criselda Mendoza-Milla

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2020)

Article Rheumatology

Severe infections in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis: a retrospective cohort study with a clinical phenotype approach

Adrian E. Caballero-Islas, Irma Hoyo-Ulloa, Annette Garcia-Castro, Andrea Hinojosa-Azaola

RHEUMATOLOGY INTERNATIONAL (2020)

Article Biology

IFN signaling and neutrophil degranulation transcriptional signatures are induced during SARS-CoV-2 infection

Bruce A. Rosa, Mushtaq Ahmed, Dhiraj K. Singh, Jose Alberto Choreno-Parra, Journey Cole, Luis Armando Jimenez-Alvarez, Tatiana Sofia Rodriguez-Reyna, Bindu Singh, Olga Gonzalez, Ricardo Carrion, Larry S. Schlesinger, John Martin, Joaquin Zuniga, Makedonka Mitreva, Deepak Kaushal, Shabaana A. Khader

Summary: The study discovered that genes associated with Interferon signaling, neutrophil degranulation, and innate immune pathways are induced in the lungs of macaques in response to SARS-CoV-2 infection, while genes related to collagen formation and regulation are reduced. Age is identified as a significant risk factor for poor prognosis and increased mortality in COVID-19.

COMMUNICATIONS BIOLOGY (2021)

Article Health Care Sciences & Services

The Scleroderma Patient-Centered Intervention Network Self-Management Program: Protocol for a Randomized Feasibility Trial

Marie-Eve Carrier, Linda Kwakkenbos, Warren R. Nielson, Claire Fedoruk, Karen Nielsen, Katherine Milette, Janet Pope, Tracy Frech, Shadi Gholizadeh, Laura Hummers, Sindhu R. Johnson, Pamela Piotrowski, Lisa Jewett, Jessica Gordon, Lorinda Chung, Dan Bilsker, Kimberly A. Turner, Julie Cumin, Joep Welling, Catherine Fortune, Catarina Leite, Karen Gottesman, Maureen Sauve, Tatiana S. Rodriguez-Reyna, Marie Hudson, Maggie Larche, Ward van Breda, Maria E. Suarez-Almazor, Susan J. Bartlett, Vanessa L. Malcarne, Maureen D. Mayes, Isabelle Boutron, Luc Mouthon, Fredrick Wigley, Brett D. Thombs

JMIR RESEARCH PROTOCOLS (2020)

Article Rheumatology

Immunosuppressive treatment in diffuse cutaneous systemic sclerosis is associated with an improved composite response index (CRISS)

Boyang Zheng, Marie Hudson, Mianbo Wang, Murray Baron, Janet E. Pope, Murray Baron, Genevieve Gyger, Maggie J. Larche, Nader A. Khalidi, Ariel Masetto, Evelyn Sutton, David Robinson, Tatiana S. Rodriguez-Reyna, Nancy Maltez, Doug Smith, Carter Thorne, Alena Ikic, Paul R. Fortin, Marvin J. Fritzler

ARTHRITIS RESEARCH & THERAPY (2020)

Article Rheumatology

Mammalian target of rapamycin is activated in the kidneys of patients with scleroderma renal crisis

Jessica Salituri, Natalie Patey, Tomoko Takano, Pierre Fiset, Sonia Del Rincon, Laeora Berkson, Murray Baron, Marie Hudson, M. Baron, M. Hudson, G. Gyger, J. Pope, M. Larche, N. Khalidi, A. Masetto, E. Sutton, D. Robinson, T. S. Rodriguez-Reyna, D. Smith, C. Thorne, P. R. Fortin, M. Fritzler

JOURNAL OF SCLERODERMA AND RELATED DISORDERS (2020)

Meeting Abstract Rheumatology

Change in Calcinosis over 1 Year Using the SCTC Radiologic Scoring System for Calcinosis of the Hands in Patients with Systemic Sclerosis

Antonia Valenzuela, Melody Chung, Tatiana S. Rodriguez-Reyna, Susanna Proudman, Murray Baron, Flavia Castelino, Vivien Hsu, Shufeng Li, David Fiorentino, Kathryn Stevens, Lorinda Chung

ARTHRITIS & RHEUMATOLOGY (2019)

Meeting Abstract Rheumatology

PANLAR STUDY GROUP CONSENSUS FOR A REPORT ON CAPILLAROSCOPY

Chiara Bertolazzi, Marwin Gutierrez, Angelica Vargas, Tatiana Sofia Rodriguez-Reyna, Hugo Sandoval, Everardo Alvarez-Hernandez, Marcelo Audisio, Eduardo Cabello, Paola Coral, Ericka Diaz, Virginia Duringa, Katirma Espejo, Selma Gallegos, Gabriela Hernandez-Molina, Blanca Herrera, Cristiane Kayser, Maria Eugenia Lara, Genessis Maldonado, Marta N. Mamani, Alejandro Nitsche, Carlos Rios-Acosta, Felix Enrique-Romanini, Maria Sormani de Fonseca, Veronica Vilela, Miguel Villarreal

JCR-JOURNAL OF CLINICAL RHEUMATOLOGY (2019)

Meeting Abstract Critical Care Medicine

Prognosis and Pulmonary Manifestations of a Cohort of Patients Positive to Th/to Autoantibodies

J. Rojas-Serrano, D. Fernandez-Badillo, D. I. Perez-Romam, L. E. Vazquez-Becerra, M. Mejia, H. N. Mateos-Toledo, L. Figueroa-Hernandez, T. S. Rodriguez-Reyna, K. Balderas-Munoz

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

Meeting Abstract Critical Care Medicine

Preservation of Lung Function Observed in a Phase 3 Randomized Controlled Trial of Tocilizumab for the Treatment of Early Systemic Sclerosis

D. Khanna, C. J. F. Lin, J. Goldin, G. Kim, M. Kuwana, Y. Allanore, A. Batalov, I. Butrimiene, P. Carreira, M. Matucci-Cerinic, O. Distler, D. Martinovic Kaliterna, C. Mihai, M. Mogensen, M. Olesinska, J. E. Pope, G. Riemekasten, T. Rodriguez-Reyna, M. J. Santos, J. van Laar, H. Spotswood, J. Siegel, A. Jahreis, D. Furst, C. P. Denton

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2019)

No Data Available