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)
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
Medicine, General & Internal
Kelly Morgan, Charlotte Woollard, Dylan Beinart, Lauren Host, Janet Roddy
Summary: The study aimed to investigate whether rituximab could stabilize or improve pulmonary function in patients with SSc-ILD. A retrospective analysis of patients who received rituximab despite progressing on conventional therapy at two tertiary centers was conducted. Results showed that all patients demonstrated stable pulmonary function testing at 1-year post-rituximab, indicating a potential role for rituximab in the treatment of SSc-ILD.
INTERNAL MEDICINE JOURNAL
(2023)
Review
Allergy
Alain Lescoat, David Roofeh, Masataka Kuwana, Robert Lafyatis, Yannick Allanore, Dinesh Khanna
Summary: Systemic sclerosis is a rheumatic disease with high mortality, and its severity is determined by fibrotic changes. The heterogeneity of the disease makes clinical trial design challenging, but improvements in classification criteria have led to better patient selection. Recent developments in classification and targeted therapies offer hope for treatment advancements.
CLINICAL REVIEWS IN ALLERGY & IMMUNOLOGY
(2023)
Review
Rheumatology
Maria Martin-Lopez, Patricia E. Carreira
Summary: Systemic sclerosis is a rare and complex disease with high morbidity and mortality, characterized by fibrosis with involvement of multiple organs. Disease management is challenging due to its heterogeneous presentation and limited controlled clinical trials. Immunosuppressive agents and antifibrotics are used to prevent disease progression.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2021)
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
Rudra P. Goswami, Animesh Ray, Moumita Chatterjee, Arindam Mukherjee, Geetabali Sircar, Parasar Ghosh
Summary: Treatment with RTX in SSc-ILD resulted in a significant improvement in both FVC and DLCO during the first year of treatment, with lower rates of infection compared to controls.
Review
Rheumatology
Martin Aringer, Gabriela Riemekasten
Summary: Systemic sclerosis is an autoimmune disease that causes skin and tissue fibrosis. Autologous stem cell transplantation has shown efficacy in systemic sclerosis and may also have benefits for interstitial lung disease. Some drugs have been approved for treating systemic sclerosis-associated interstitial lung disease.
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
(2021)
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)
Article
Dermatology
Jun Omatsu, Ryosuke Saigusa, Takuya Miyagawa, Yuki Fukui, Satoshi Toyama, Kentaro Awaji, Tetsuya Ikawa, Yuta Norimatsu, Ayumi Yoshizaki, Shinichi Sato, Yoshihide Asano
Summary: The expression levels of S100A12 are significantly elevated in systemic sclerosis (SSc) patients, especially in lesional skin and bulk skin. Serum S100A12 levels are correlated with the severity of skin involvement and the presence of interstitial lung disease in SSc patients.
EXPERIMENTAL DERMATOLOGY
(2021)
Article
Immunology
Naoko Takamura, Ludivine Renaud, Willian Abraham da Silveira, Carol Feghali-Bostwick
Summary: Systemic sclerosis is an autoimmune disease characterized by vasculopathy and excessive fibrosis, with limited treatment options available. Research suggests that abnormal regulation of MCHR1 may play a role in the pathogenesis of SSc, and inhibiting MCHR1 could be a novel therapeutic strategy.
FRONTIERS IN IMMUNOLOGY
(2021)
Article
Immunology
Ming-Li Zou, Ying-Ying Teng, Zhong-hua Chen, Si-Yu Liu, Yuan Jia, Kai-Wen Zhang, Jun-Jie Wu, Zheng-Dong Yuan, Xiao-Yu Tang, Shun Yu, Jun-Xing Ye, Xia Li, Xiao-Jin Zhou, Feng-Lai Yuan
Summary: Skin fibrosis is a common pathological feature of various diseases. The urokinase-type plasminogen activator (uPA) system, including uPA, urokinase plasminogen activator receptor (uPAR), and plasminogen activator inhibitor-1 (PAI-1), is upregulated in fibrotic diseases. The successful binding of uPA and uPAR activates the downstream peroxisome proliferator-activated receptor (PPAR) signaling pathway, reducing the proliferation, migration, and contraction of disease-derived fibroblasts and alleviating skin fibrosis. However, increased or robust upregulation of PAI-1 inhibits these effects, suggesting the involvement of PAI-1 in skin fibrosis.
FRONTIERS IN IMMUNOLOGY
(2022)
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
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)
Article
Rheumatology
Alain Lescoat, Doerte Huscher, Nils Schoof, Paolo Airo, Jeska De Vries-Bouwstra, Gabriela Riemekasten, Eric Hachulla, Andrea Doria, Edoardo Rosato, Nicolas Hunzelmann, Carlomaurizio Montecucco, Armando Gabrielli, Anna-Maria Hoffmann-Vold, Oliver Distler, Jennifer Ben Shimol, Maurizio Cutolo, Yannick Allanore
Summary: The prevalence and characteristics of SSc-associated interstitial lung disease (SSc-ILD) vary between geographical regions. This study aimed to explore the differences in prevalence, phenotype, treatment, and prognosis in patients with SSc-ILD from different regions in the EUSTAR database. The results showed significant variations in clinical characteristics, survival rates, and treatment approaches among different regions.
