Review
Immunology
Claire F. Beesley, Nina R. Goldman, Taher E. Taher, Christopher P. Denton, David J. Abraham, Rizgar A. Mageed, Voon H. Ong
Summary: Systemic sclerosis (SSc) is an immune-mediated rheumatic disease characterized by excessive extracellular matrix deposition. B cells play a fundamental role in the pathogenesis and development of SSc, as they infiltrate lesional sites and produce profibrotic cytokines. B cell counts are increased in SSc patients and show differences in various B cell compartments. B cell signaling is impaired in SSc patients, and B cell depletion therapy has shown therapeutic benefits.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Immunology
Maheswari Muruganandam, Angie Ariza-Hutchinson, Rosemina A. Patel, Wilmer L. Sibbitt
Summary: Systemic sclerosis is a complex autoimmune disease characterized by vascular damage, inflammation, and fibrosis. Biomarkers play an important role in understanding the disease process and potential therapeutic targets. Anti-nuclear antibodies are classical biomarkers, while other proteins and pathways are also implicated. The use of biomarker panels combined with advanced analysis techniques can help determine disease activity and treatment response.
JOURNAL OF INFLAMMATION RESEARCH
(2023)
Review
Immunology
Benjamin Thoreau, Benjamin Chaigne, Luc Mouthon
Summary: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by fibrosis, vasculopathy, and autoimmunity. Recent studies have shown the significant role of B-cells in SSc, with disrupted subpopulations and activated phenotype. B-cells also exhibit impaired regulatory capacities and contribute to fibrosis through the production of autoantibodies and cytokines. Targeting B-cells may be a potential treatment for early-stage SSc patients with severe organ damage.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Immunology
Ayumi Yoshizaki, Takemichi Fukasawa, Satoshi Ebata, Asako Yoshizaki-Ogawa, Shinichi Sato
Summary: This article outlines the role of B cells in the development of systemic sclerosis (SSc), including their involvement in autoimmune abnormalities, pro-inflammatory actions, and inhibitory functions. Studies have found B-cell depletion therapy to be effective for SSc.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Rheumatology
Qiang Li, Laura Wallace, Padmaja Patnaik, Margarida Alves, Martina Gahlemann, Veronika Kohlbrenner, Christina Raabe, Jocelyn R. Wang, Elizabeth M. Garry
Summary: The study found low prevalence estimates and incidence rates for SSc and SSc-ILD in the US, with newly diagnosed SSc-ILD patients receiving more immunosuppressive therapy and having more comorbidities compared to newly diagnosed SSc patients.
Review
Biochemistry & Molecular Biology
Joe E. Mouawad, Carol Feghali-Bostwick
Summary: Systemic sclerosis, also known as scleroderma, is an autoimmune disorder that affects the connective tissues and has a high mortality rate. Fibrosis, particularly in the lungs, is a hallmark of the disease and is currently the leading cause of death. Understanding the molecular mechanisms involved in lung fibrosis is essential for developing potential therapies to improve patient outcomes and quality of life.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Immunology
Fatemeh Vafashoar, Kazem Mousavizadeh, Hadi Poormoghim, Amir Haghighi, Salar Pashangzadeh, Nazanin Mojtabavi
Summary: The study found that progesterone treatment increased collagen content in fibrotic and normal lung tissues, as well as increased alpha-SMA and TGF-beta in fibrotic lung tissues while decreasing MMP9. Furthermore, progesterone treatment also decreased the gene expression of Col1a2, Ctgf, and End1 in bleomycin-injured lung tissues.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Bo Broens, Conny J. van der Laken, Gerben J. C. Zwezerijnen, Esther J. Nossent, Lilian J. Meijboom, Julia Spierings, Jeska K. de Vries-Bouwstra, Jacob M. van Laar, Alexandre E. Voskuyl
Summary: Positron emission tomography (PET) is a promising technique for evaluating systemic sclerosis associated interstitial lung disease (SSc-ILD), particularly in severe diffuse cutaneous SSc (dcSSc) patients eligible for autologous hematopoietic stem cell transplantation (aHSCT). This article discusses the potential benefits of using PET in early severe dcSSc and ILD patients in the context of aHSCT, as well as the potential value of other PET tracers in ILD assessment and understanding the mechanisms of aHSCT in the lung.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Chemistry, Medicinal
Martina Orlandi, Laura Antonia Meliante, Arianna Damiani, Lorenzo Tofani, Cosimo Bruni, Serena Guiducci, Marco Matucci-Cerinic, Silvia Bellando-Randone, Sara Tomassetti
Summary: The use of bronchoalveolar lavage (BAL) in the evaluation of systemic sclerosis (SSc) interstitial lung disease (ILD) remains controversial. Studies have found a positive correlation between BAL cytology and lung function, as well as high-resolution computed tomography (HRCT) findings. Cytokines, chemokines, growth factors, coagulation factors, and eicosanoids have been shown to be present in higher quantities in SSc-ILD patients and may be related to more severe pulmonary disease. However, there is no consensus regarding the role of BAL cellularity as a predictor of mortality.
Article
Immunology
Eleanor Valenzi, Tracy Tabib, Anna Papazoglou, John Sembrat, Humberto E. Trejo Bittar, Mauricio Rojas, Robert Lafyatis
Summary: IPF and SSc-ILD exhibit differences in cell types and pathways, providing new insights into the diseases.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Immunology
Konstantinos Melissaropoulos, George Iliopoulos, Lazaros Sakkas, Dimitrios Daoussis
Summary: Systemic sclerosis is a rare fibrotic rheumatic disease that is associated with psychological distress, skin involvement, and internal organ damage. The understanding of its complex pathogenesis is incomplete and current therapeutic algorithms are not optimal. B cells play a significant role in systemic sclerosis, with imbalances and abnormal receptor signaling being key factors of interest.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Junsuk Ko, Maria Noviani, Vasuki Ranjani Chellamuthu, Salvatore Albani, Andrea Hsiu Ling Low
Summary: Systemic sclerosis is an autoimmune disease with unclear pathophysiology, but vascular abnormalities play a significant role in its development.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Chemistry, Medicinal
Barbara Ruaro, Marco Confalonieri, Marco Matucci-Cerinic, Francesco Salton, Paola Confalonieri, Mario Santagiuliana, Gloria Maria Citton, Elisa Baratella, Cosimo Bruni
Summary: Systemic sclerosis patients often suffer from interstitial lung disease, with early diagnosis playing a pivotal role in improving prognosis. Treatment strategies typically involve a combination of immunosuppressants and targeted biological therapies based on disease severity and progression risk. Hematopoietic autologous stem cell transplantation has shown benefits for progressive SSc patients, while lung transplantation is considered for refractory cases of SSc-ILD.
Review
Immunology
Margherita Giannini, Benjamin Ellezam, Valerie Leclair, Frederic Lefebvre, Yves Troyanov, Marie Hudson, Jean-Luc Senecal, Bernard Geny, Oceane Landon-Cardinal, Alain Meyer
Summary: Systemic sclerosis and autoimmune myositis are associated with decreased quality of life and increased mortality. Recent findings have identified a new disease subset, called scleromyositis, within both diseases, which has important implications for patient management and understanding of the disease pathophysiology.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Rheumatology
Burcu Ayoglu, Michele Donato, Daniel E. Furst, Leslie J. Crofford, Ellen Goldmuntz, Lynette Keyes-Elstein, Judith James, Susan Macwana, Maureen D. Mayes, Peter McSweeney, Richard A. Nash, Keith M. Sullivan, Beverly Welch, Ashley Pinckney, Rong Mao, Lorinda Chung, Purvesh Khatri, Paul J. Utz
Summary: Results from the SCOT clinical trial showed that HSCT had significant benefits over CTX in patients with systemic sclerosis. The objective of this study was to test the hypothesis that transplantation stabilizes the autoantibody repertoire in patients with favorable clinical outcomes. Analysis of autoantibody profiles revealed significant differences between HSCT and CTX-treated patients, suggesting that HSCT alters the autoantibody repertoire while CTX treatment does not.
ANNALS OF THE RHEUMATIC DISEASES
(2023)