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
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)
Article
Medicine, General & Internal
Paulina Szczepanik-Kulak, Malgorzata Michalska-Jakubus, Malgorzata Kowal, Dorota Krasowska
Summary: This study analyzed the clinical significance of IL-1 family cytokines in localized scleroderma (LoS) patients and compared their levels before and after treatment. The results showed significant changes in IL-1 alpha and IL-33 concentrations after treatment in the LoS group. There were also significant negative correlations between IL-1 alpha concentration and PGA-A index after treatment.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Endocrinology & Metabolism
Qian Wang, Wenjing Ye, Fangfang Chen, Minrui Liang, Weiguo Wan, Wenyu Guo, Hejian Zou, Yu Xue
Summary: The study suggests that inhibiting the IL-6 transmitter signal with TJ301 may be a novel approach to treat systemic sclerosis (SSc).
JOURNAL OF BIOLOGICAL REGULATORS AND HOMEOSTATIC AGENTS
(2023)
Article
Dermatology
Xuefen Wu, Bingxia Ming, Tong Wu, Rongfen Gao, Peng Hu, Jungen Tang, Jixin Zhong, Fang Zheng, Lingli Dong
Summary: IL-33/ST2 axis plays a crucial role in the fibrotic disorder of SSc by promoting fibroblast activation, and IL-33/ST2 blockade could be a potential novel strategy to inhibit fibrosis progression in SSc patients.
JOURNAL OF DERMATOLOGICAL SCIENCE
(2022)
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)
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)
Review
Rheumatology
Ioannis Sagonas, Dimitrios Daoussis
Summary: Serotonin plays a crucial role in fibrosis and vasculopathy in systemic sclerosis, suggesting the need for further exploration of its potential role in the disease.
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
Devis Benfaremo, Silvia Agarbati, Matteo Mozzicafreddo, Chiara Paolini, Silvia Svegliati, Gianluca Moroncini
Summary: Systemic sclerosis, also known as scleroderma or SSc, is a highly varied disease in terms of clinical presentation, disease progression, and treatment response. Recent advancements in skin molecular profiling technologies have enabled the identification of patient subgroups based on gene expression analysis. This narrative review provides an overview of the current status of skin gene expression analysis and emphasizes the benefits of stratifying patients based on their gene signatures. This stratification has the potential to lead to a precision medicine approach in the management of SSc.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Rheumatology
Barbara Russo, Julia Borowczyk, Pietro Cacialli, Philippe Moguelet, Marie-Elise Truchetet, Ali Modarressi, Nicolo C. Brembilla, Julien Bertrand, Wolf-Henning Boehncke, Carlo Chizzolini
Summary: IL-25 plays a role in regulating skin fibrosis, and its decreased expression in systemic sclerosis may contribute to the excessive accumulation of extracellular matrix and progress of fibrosis.
Review
Immunology
Lanxuan Wei, David Abraham, Voon Ong
Summary: IL-17 is a pro-inflammatory cytokine that plays an important role in host defense. It activates transcription and stabilizes mRNAs through multiple pathways, mainly acting on stromal cells to promote the secretion of other immunomodulatory factors and support the growth and differentiation of immune cells. IL-17 has a dual role in immune reactions and inflammatory diseases, and it also plays a significant role in autoimmune diseases.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Vincent Cottin, Sophie Larrieu, Loic Boussel, Salim Si-Mohamed, Fabienne Bazin, Sebastien Marque, Jacques Massol, Francoise Thivolet-Bejui, Lara Chalabreysse, Delphine Maucort-Boulch, Stephane Jouneau, Eric Hachulla, Julien Chollet, Mouhamad Nasser
Summary: This study in France investigated the clinical characteristics, epidemiology, survival estimates, and healthcare resource utilization in patients with SSc-ILD. It found a high prevalence of SSc-ILD, with patients having a mean age of 60.6 years and significant healthcare costs associated with the disease.
FRONTIERS IN MEDICINE
(2021)
Article
Biochemistry & Molecular Biology
Anca Cardoneanu, Alexandra Maria Burlui, Luana Andreea Macovei, Ioana Bratoiu, Patricia Richter, Elena Rezus
Summary: Systemic sclerosis (SS) is a chronic autoimmune disorder characterized by cutaneous and systemic clinical manifestations. Interleukin-6 (IL-6) plays a critical role in the development of vascular damage and fibrosis in SS. Tocilizumab, an anti-IL-6 therapy, shows promising results in improving skin and lung damage in SS patients.
Review
Dermatology
Yusheng Zhang, Narges Maskan Bermudez, Brianna Sa, Andrea D. Maderal, Joaquin J. Jimenez
Summary: This article reviews key studies and findings to comprehensively understand how dysregulated epigenetic mechanisms contribute to the development of SLE, SSc, and DM.
EXPERIMENTAL DERMATOLOGY
(2023)