Review
Biochemistry & Molecular Biology
Suhee Kim, Hee Jin Park, Sang-Il Lee
Summary: Systemic sclerosis, also known as scleroderma, is an autoimmune disease that affects multiple organs. Recent research has found a connection between the gut microbiota and scleroderma, suggesting novel therapeutic possibilities. This review summarizes the current understanding of the molecular mechanisms of scleroderma and the role of the microbiome, as well as discusses potential treatment interventions.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Immunology
Zongzhi Jiang, Ziyi Wang, Xiaojing Wei, Xue-Fan Yu
Summary: This review article summarizes the role and application of inflammatory markers in amyotrophic lateral sclerosis (ALS). Inflammatory molecules and proteins play a key role in the pathogenesis of ALS and may serve as indicators for predicting patient survival and evaluating treatment response. Furthermore, inflammatory markers have the potential to be used as new therapeutic targets and strategies to expand the therapeutic interventions for ALS.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Rheumatology
Tatiana Nevskaya, Janet E. Pope, Matthew A. Turk, Jenny Shu, April Marquardt, Frank van den Hoogen, Dinesh Khanna, Jaap Fransen, Marco Matucci-Cerinic, Murray Baron, Christopher P. Denton, Sindhu R. Johnson
Summary: Research findings suggest that systemic sclerosis (SSc) subset classification systems are primarily based on the extent of skin involvement and/or SSc-specific autoantibodies, nailfold capillary patterns, and molecular, genomic, and cellular patterns. While some classification systems have prognostic value for clinical phenotype, severity, and mortality, only subsetting by gene expression signatures in tissue samples has been linked to response to therapy.
JOURNAL OF RHEUMATOLOGY
(2021)
Review
Rheumatology
Xiongjie Bi, Tingting Mills, Minghua Wu
Summary: This review provides an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis, focusing on the most recently generated and investigated animal models that delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function, and pulmonary artery hypertension (PAH).
CURRENT OPINION IN RHEUMATOLOGY
(2023)
Article
Immunology
Jiahui Jin, Yifan Liu, Qinyu Tang, Xin Yan, Miao Jiang, Xu Zhao, Jie Chen, Caixia Jin, Qingjian Ou, Jingjun Zhao
Summary: This study identified tissue-specific expressed hub genes associated with systemic sclerosis (SSc) using integrated bioinformatics. A ceRNA regulatory network and potential targeted drugs were also discovered. A total of 254 differentially expressed genes were identified in SSc, and four hub genes (SERPINE1, CCL2, IL6, and ISG15) were found to be significantly upregulated in SSc patients. Additionally, several RNA regulatory pathways and potential drugs associated with these genes were identified.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Medicine, General & Internal
Anna Bohdziewicz, Katarzyna Karina Pawlik, Magdalena Maciejewska, Mariusz Sikora, Rosanna Alda-Malicka, Joanna Czuwara, Lidia Rudnicka
Summary: Systemic sclerosis is a complex autoimmune disease characterized by vasculopathy and fibrosis. Current therapeutic options are insufficient, but research on novel targets shows potential for new treatment options in the future.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Cell Biology
Nancy Tarantino, Ileana Canfora, Giulia Maria Camerino, Sabata Pierno
Summary: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by progressive loss of motor neurons and muscle function. The disease involves a complex cascade of events among motor neurons, glia, and muscles, and is associated with mitochondrial dysfunction and other mechanisms. Currently, there are limited treatment options, and there is a need to find effective treatments.
Review
Veterinary Sciences
Monique Ribeiro de Lima, Daiani Cotrim de Paiva Campbell, Mariana Rietmann da Cunha-Madeira, Barbara Cristina Marcollino Bomfim, Jackline de Paula Ayres-Silva
Summary: Research on the effects of radiation exposure expanded significantly after World War II, with scientists conducting experiments on animals to understand more about radiation. To ensure the well-being of animals in research, scientists developed scoring systems to assess their condition. Implementing common rules is important for accurate research and collaboration among scientists.
VETERINARY SCIENCES
(2023)
Article
Rheumatology
Bohdana Doskaliuk, Liubomyr Zaiats
Summary: This study aimed to investigate the effects of prolonged exposure to NaClO on lung tissues in laboratory animals. The findings showed that long-term exposure to NaClO resulted in significant histopathological changes in lung tissues, including damage to the pulmonary microcirculation and disruption in the structure of endothelial cells.
RHEUMATOLOGY INTERNATIONAL
(2023)
Article
Green & Sustainable Science & Technology
Martin Jung, Myroslava Lesiv, Eleanor Warren-Thomas, Dmitry Shchepashchenko, Linda See, Steffen Fritz
Summary: Restoring forests is a global policy priority, but the type of forest being restored is often overlooked, despite its impact on biodiversity and livelihoods. This study used a map of forest management types to evaluate the extent of managed forests in recent tree cover gains worldwide.
NATURE SUSTAINABILITY
(2023)
Review
Clinical Neurology
Yinan Zhang, Jeffrey Atkinson, Christin E. Burd, Jennifer Graves, Benjamin M. Segal
Summary: Multiple sclerosis (MS) tends to progress during middle age or beyond, and the number of older adults with MS is increasing. Studies have shown that biological aging is accelerated in people with MS compared with healthy controls, and it interacts with pathogenic pathways to drive disability accumulation. Future research focusing on aging-related pathways may lead to the development of novel biomarkers and therapies to slow neurological decline in progressive MS patients.
MULTIPLE SCLEROSIS JOURNAL
(2023)
Review
Biology
Angelica Mandujano, Melissa Golubov
Summary: Systemic sclerosis is an autoimmune disease characterized by inflammation, vasculopathy, and fibrosis. Despite the development of various animal models, none fully replicate all aspects of the disease. Nailfold capillaroscopy, widely used in humans, has potential applications in animal models for studying vasculopathy and developing novel treatment options.
Review
Medicine, Research & Experimental
Hao Ren, Licong Liu, Yangfan Xiao, Yaqian Shi, Zhuotong Zeng, Yan Ding, Puyu Zou, Rong Xiao
Summary: Systemic sclerosis is an autoimmune disease characterized by immune dysfunction, vascular system dysfunction, and tissue fibrosis. Early diagnosis and rational treatment are crucial for improving patient survival and quality of life.
BIOMEDICINE & PHARMACOTHERAPY
(2023)
Review
Immunology
Yongmei Liu, Linlin Cheng, Haoting Zhan, Haolong Li, Xiaomeng Li, Yuan Huang, Yongzhe Li
Summary: Noncoding RNAs (ncRNAs) make up more than 90% of the RNAs in the human genome. Recent studies have shown that ncRNAs are not just junk products, but play a role as functional regulatory molecules in important processes such as chromosomal modifications, mRNA splicing and stability, translation, and signaling pathways. Abnormal expression of ncRNAs has been observed in cancer and autoimmune diseases like systemic sclerosis (SSc), making them potential biomarkers and therapeutic targets for the diagnosis and treatment of SSc. However, our understanding of the functions and mechanisms of ncRNAs in SSc is still incomplete.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Biotechnology & Applied Microbiology
Erica Mulcaire-Jones, Andrea Hsiu Ling Low, Robyn Domsic, Michael L. Whitfield, Dinesh Khanna
Summary: Systemic sclerosis (SSc) is a severe autoimmune disease that causes inflammation and fibrosis of the skin and internal organs. Limited therapeutic options are available for SSc patients. There are ongoing studies exploring biologic therapies targeting the underlying pathogenesis of SSc.
EXPERT OPINION ON BIOLOGICAL THERAPY
(2023)
Article
Rheumatology
Yossra Suliman, Suzanne Kafaja, Sunny J. Oh, Mohamed Alemam, Gianluca Bagnato, Giuseppina Abignano, Ram Raj Singh, Gillian Barlow, Xiaochen Liu, Isela Valera, Walter Morales, Ali Rezaie, Mark Pimentel, Francesco Del Galdo, Daniel E. Furst
Summary: The study shows that elevated anti-vinculin antibody levels are common in patients with SSc and suggests a potential link between increased anti-vinculin levels and gastrointestinal symptoms.
CLINICAL RHEUMATOLOGY
(2021)
Article
Rheumatology
Giuseppina Abignano, Gianna Angela Mennillo, Giovanni Lettieri, Duygu Temiz Karadag, Antonio Carriero, Angela Anna Padula, Francesco Del Galdo, Dinesh Khanna, Salvatore D'Angelo
Summary: The study demonstrated that SSc patients with impaired esophageal transit scintigraphy findings have higher GIT 2.0 reflux scores, while esophageal emptying activity is negatively correlated with reflux scores.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Rheumatology
Christopher W. Wasson, Rebecca L. Ross, Ruth Morton, Jamel Mankouri, Francesco Del Galdo
Summary: This study identified a novel role for CLIC4 in SSc myofibroblast activation, highlighting its similarities with cancer-associated fibroblasts. Inhibition of CLIC4 resulted in reduced expression of myofibroblast markers in SSc fibroblasts, indicating a direct role for CLIC4 in disease pathogenesis. CLIC4 may serve as a potential therapeutic target for SSc.
Article
Rheumatology
Raluca B. Dumitru, Lesley-Anne Bissell, Bara Erhayiem, Graham Fent, Ananth Kidambi, Peter Swoboda, Giuseppina Abignano, Helena Donica, Agata Burska, John P. Greenwood, John Biglands, Francesco Del Galdo, Sven Plein, Maya H. Buch
Summary: The subclinical phenotype of SSc primary heart involvement is characterized by myocardial microvasculopathy, diffuse and focal myocardial fibrosis while retaining preserved myocardial contractile function. This phenotype is associated with high-sensitivity troponin I, N-terminal pro-brain natriuretic peptide, SSc disease severity, and complicated peripheral vasculopathy, providing insights into potential underlying pathophysiological processes and risk factors for SSc-pHI.
Article
Rheumatology
Rebecca L. Ross, Clarissa Corinaldesi, Gemma Migneco, Ian M. Carr, Agne Antanaviciute, Christopher W. Wasson, Antonio Carriero, Jorg H. W. Distler, Steve Holmes, Yasser M. El-Sherbiny, Clive S. McKimmie, Francesco Del Galdo
Summary: Human pDC play a significant role in inflammation and immune-driven skin fibrosis, which can be effectively blocked by BDCA2-targeting, providing direct evidence supporting the development of attenuation of pDC function as a therapeutic application for SSc.
ANNALS OF THE RHEUMATIC DISEASES
(2021)
Article
Biochemistry & Molecular Biology
Clarissa Corinaldesi, Rebecca L. Ross, Giuseppina Abignano, Cristina Antinozzi, Francesco Marampon, Luigi di Luigi, Maya H. Buch, Valeria Riccieri, Andrea Lenzi, Clara Crescioli, Francesco Del Galdo
Summary: Sildenafil treatment is associated with lower serum levels of CXCL10, which is elevated in patients with systemic sclerosis (SSc). Serum CXCL10 is strongly correlated with muscle involvement severity and CK levels in SSc patients, suggesting its potential involvement in muscle damage. In vitro studies show that sildenafil dose-dependently reduces CXCL10 release by myocytes and inhibits phosphorylation of key signaling molecules, supporting the idea that PDE5i could be a therapeutic strategy for preventing and treating muscle damage in SSc.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Cell Biology
Christopher W. Wasson, Begona Caballero-Ruiz, Justin Gillespie, Emma Derrett-Smith, Jamel Mankouri, Christopher P. Denton, Gianluca Canettieri, Natalia A. Riobo-Del Galdo, Francesco Del Galdo
Summary: This study reveals that CLIC4 expression is regulated by the signaling crosstalk between TGF-beta, Wnt-3a, and Hh, and that it strongly correlates with the expression of the pro-fibrotic marker alpha-smooth muscle actin. Further investigations demonstrate that inhibiting CLIC4 can reverse morphogen-dependent fibroblast activation.
Article
Dermatology
Giuseppina Abignano, Lorraine Green, Sookhoe Eng, Paul Emery, Francesco Del Galdo
Summary: This study aimed to determine whether dynamic-optical coherence tomography (D-OCT) can provide information on microvasculopathy in patients with systemic sclerosis compared to the current gold standard, nailfold video-capillaroscopy (NVC). In conclusion, D-OCT provided qualitative and quantitative information on nailfold microvasculopathy, showing a correlation between microvascular flow density and NVC scores. The development of D-OCT as a standardized imaging technique could provide a quantitative outcome measure in clinical trials and practice.
JOURNAL OF INVESTIGATIVE DERMATOLOGY
(2022)
Article
Rheumatology
Christopher P. Denton, Francesco Del Galdo, Dinesh Khanna, Madelon C. Vonk, Lorinda Chung, Sindhu R. Johnson, John Varga, Daniel E. Furst, Jane Temple, Chiara Zecchin, Eszter Csomor, Amy Lee, Nicolas Wisniacki, Shaun M. Flint, Juliet Reid
Summary: This study aimed to explore the feasibility of inhibiting SSc progression using the anti-OSM monoclonal antibody GSK2330811. The results showed that GSK2330811 treatment did not demonstrate significant mechanistic effects on inflammation or fibrosis, and there were no meaningful differences between GSK2330811 and placebo for any clinical efficacy endpoints. Furthermore, adverse events related to decreases in hemoglobin and platelet count were observed in the 300 mg dose group.
Article
Rheumatology
Paola Cipriani, Piero Ruscitti, Ilenia Di Cola, Marta Vomero, Franca Abbruzzese, Elena Di Nino, Rebecca Ross, Francesco Del Galdo, Roberto Giacomelli
Summary: CD248 has anti-angiogenic effects in vitro by reducing tube formation and inducing apoptosis in endothelial cells. Increased expression of CD248 in SSc may contribute to the observed microvascular rarefaction at the tissue level. Our results suggest a pathogenic role for CD248-MMNR-2 in SSc.
Editorial Material
Rheumatology
Silvia Laura Bosello, Edward M. Vital, Francesco Del Galdo
Article
Dermatology
Anto J. U. K. John, Francesco Del Galdo, Rodney Gush, Peter R. R. Worsley
Summary: This study aimed to evaluate non-invasive tools for assessing the mechanical and functional properties of skin in different body regions. Significant differences in these properties were observed between skin sites. The results suggest that these parameters can be reliably measured and used to assess and monitor changes in the skin during health and disease.
SKIN RESEARCH AND TECHNOLOGY
(2023)
Review
Rheumatology
Enrico De Lorenzis, Christopher William Wasson, Francesco Del Galdo
Summary: The alveolar epithelial-to-mesenchymal transition is a process that involves the conversion of differentiated epithelial cells into mesenchymal-like cells through functional and morphological changes. This transition can indirectly contribute to lung fibrosis by stimulating surrounding cells, as well as directly increasing pulmonary fibroblasts and extracellular matrix deposition. Directly demonstrating this transition in scleroderma-related interstitial lung disease is challenging due to technical limitations and limited availability of lung tissue samples. However, pre-clinical and clinical evidence suggests that epithelial-to-mesenchymal transition may play a role in the development and progression of lung fibrosis in this disease.
JOURNAL OF SCLERODERMA AND RELATED DISORDERS
(2023)
Article
Rheumatology
Raluca B. Dumitru, Lesley-Anne Bissell, Bara Erhayiem, Graham Fent, Ananth Kidambi, Giuseppina Abignano, John P. Greenwood, John Biglands, Francesco Del Galdo, Sven Plein, Maya H. Buch
Summary: This study aimed to investigate the progression of subclinical systemic sclerosis (SSc) primary heart involvement over time. The results showed that a small number of patients had an increase or new findings of cardiac abnormalities, but most of the abnormalities remained stable during follow-up.
ACR OPEN RHEUMATOLOGY
(2023)
Article
Rheumatology
Christopher P. Denton, Enrico De Lorenzis, Elen Roblin, Nina Goldman, Begonya Alcacer-Pitarch, Emma Blamont, Maya Buch, Maresa Carulli, Caroline Cotton, Francesco del Galdo, Emma Derrett-Smith, Karen Douglas, Sue Farrington, Kim Fligelstone, Luke Gompels, Bridget Griffiths, Ariane Herrick, Michael Hughes, Clare Pain, Georgina Pantano, John Pauling, Athiveeraramapandian Prabu, Nuala O'Donoghue, Elisabetta Renzoni, Jeremy Royle, Muditha Samaranayaka, Julia Spierings, Aoife Tynan, Louise Warburton, Voon Ong
Summary: This guideline provides practical roadmap for the management of SSc, covering general approaches to management as well as treatment of specific complications.
RHEUMATOLOGY ADVANCES IN PRACTICE
(2022)
