Article
Critical Care Medicine
Anna -Maria Hoffmann-Vold, Cathrine Brunborg, Paolo Airo, Lidia P. Ananyeva, Laszlo Czirjak, Serena Guiducci, Eric Hachulla, Mengtao Li, Carina Mihai, Gabriela Riemekasten, Petros P. Sfikakis, Gabriele Valentini, Otylia Kowal-Bielecka, Yannick Allanore, Oliver Distler
Summary: This study aimed to test whether enrichment strategies from clinical trials for progressive systemic sclerosis-associated interstitial lung disease (SSc-ILD) affect efficacy, representativeness, and feasibility. The researchers applied the inclusion criteria of major recent SSc-ILD trials to patients with SSc-ILD from the European Scleroderma Trials and Research (EUSTAR) database. The results showed that patient populations that fulfilled different study inclusion criteria significantly differed in various clinical parameters, and the application of enrichment criteria did not have a significant impact on ILD progression.
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.
Article
Rheumatology
David Roofeh, Kevin K. Brown, Ella A. Kazerooni, Donald Tashkin, Shervin Assassi, Fernando Martinez, Athol U. Wells, Ganesh Raghu, Christopher P. Denton, Lorinda Chung, Anna-Maria Hoffmann-Vold, Oliver Distler, Kerri A. Johannson, Yannick Allanore, Eric L. Matteson, Leticia Kawano-Dourado, John D. Pauling, James R. Seibold, Elizabeth R. Volkmann, Simon L. F. Walsh, Chester Oddis, Eric S. White, Shaney L. Barratt, Elana J. Bernstein, Robyn T. Domsic, Paul F. Dellaripa, Richard Conway, Ivan Rosas, Nitin Bhatt, Vivien Hsu, Francesca Ingegnoli, Bashar Kahaleh, Puneet Garcha, Nishant Gupta, Surabhi Khanna, Peter Korsten, Celia Lin, Stephen C. Mathai, Vibeke Strand, Tracy J. Doyle, Virginia Steen, Donald F. Zoz, Juan Ovalles-Bonilla, Ignasi Rodriguez-Pinto, Padmanabha D. Shenoy, Andrew Lewandoski, Elizabeth Belloli, Alain Lescoat, Vivek Nagaraja, Wen Ye, Suiyuan Huang, Toby Maher, Dinesh Khanna
Summary: This study aimed to establish a framework by which experts define disease subsets in systemic sclerosis associated interstitial lung disease (SSc-ILD). The results showed that using the proposed conceptual framework, international experts were able to achieve a consensus on classifying SSc-ILD patients along the dimensions of disease severity, risk of progression and progression over time.
Article
Rheumatology
Cosimo Bruni, Mariaelena Occhipinti, Michael Pienn, Gianna Camiciottoli, Maurizio Bartolucci, Silvia Laura Bosello, Christian Payer, Zoltan Balint, Anna Rita Larici, Alessandra Tottoli, Lorenzo Tofani, Enrico De Lorenzis, Gemma Lepri, Silvia Bellando-Randone, Amelia Spinella, Dilia Giuggioli, Francesco Masini, Giovanna Cuomo, Federico Lavorini, Stefano Colagrande, Horst Olschewski, Marco Matucci-Cerinic
Summary: Recent advancements have made it possible to quantitatively assess lung vascular and parenchymal changes in thoracic CT images using automated software tools. In this study, the researchers investigated the vessel parameters of patients with SSc and correlated them with interstitial lung disease (ILD) features. The results showed that the CT vessel parameters increased in parallel with ILD extent and functional impairment, suggesting that they may serve as a biomarker of SSc-ILD severity.
Review
Biochemistry & Molecular Biology
Patricia Richter, Anca Cardoneanu, Nicoleta Dima, Ioana Bratoiu, Ciprian Rezus, Alexandra Maria Burlui, Damiana Costin, Luana Andreea Macovei, Elena Rezus
Summary: Interstitial lung disease (ILD) is a serious manifestation of connective tissue diseases (CTD) that requires thorough evaluation and treatment. The prevalence of ILD in systemic lupus erythematosus (SLE) is still debated, but it is crucial to exclude an overlap syndrome for an accurate diagnosis. Increasing the identification of SLE-associated ILD cases is important, and various treatment options are being proposed. Systemic sclerosis (SSc)-associated ILD is a leading cause of mortality and should be investigated in all SSc patients. Progress has been made in the treatment of ILD, with tyrosine kinases inhibitor Nintedanib showing promising results in reducing the progression rate compared to placebo.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Alexandra Nagy, Erik Palmer, Lorinc Polivka, Noemi Eszes, Krisztina Vincze, Eniko Barczi, Aniko Bohacs, Adam Domonkos Tarnoki, David Laszlo Tarnoki, Gyoergy Nagy, Emese Kiss, Pal Maurovich-Horvat, Veronika Mueller
Summary: This study evaluated the clinical characteristics and predictors of lung function changes in SSc-ILD patients treated in a real-world setting. The results showed that untreated patients had the most prominent functional decline, and a normal body mass index was associated with a significant risk of deterioration. The majority of patients improved or were stable during follow-up.
Article
Biochemistry & Molecular Biology
Caterina Oriana Aragona, Antonio Giovanni Versace, Carmelo Ioppolo, Daniela La Rosa, Rita Lauro, Maria Concetta Tringali, Simona Tomeo, Guido Ferlazzo, William Neal Roberts, Alessandra Bitto, Natasha Irrera, Gianluca Bagnato
Summary: This review discusses recent advancements in the treatment of systemic sclerosis-associated interstitial lung disease, including new molecular targets and approved drugs to slow disease progression. The focus is on personalized treatment strategies based on emerging pathogenic features of the disease.
Article
Health Care Sciences & Services
Chiara Pellicano, Marzia Miglionico, Laura Romaggioli, Amalia Colalillo, Lorenzo Vantaggio, Cecilia Napodano, Cinzia Calla, Francesca Gulli, Mariapaola Marino, Umberto Basile, Edoardo Rosato
Summary: This study found that SSc patients have increased levels of CH50 and serum C2 compared to healthy controls. These findings may be associated with disease severity and the severity of skin and lung fibrosis in SSc patients.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Article
Medicine, General & Internal
Hye Jin Jang, Ala Woo, Song Yee Kim, Seung Hyun Yong, Youngmok Park, Kyungsoo Chung, Su Hwan Lee, Ah Young Leem, Sang Hoon Lee, Eun Young Kim, Ji Ye Jung, Young Ae Kang, Young Sam Kim, Moo Suk Park
Summary: This study aimed to identify risk factors for mortality and compare the clinical characteristics of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). The study found that approximately 10% of SSc-ILD patients showed disease progression, and ILD was detected within less than five years from the first visit. Therefore, careful monitoring of symptoms and signs from an early stage and long-term surveillance are necessary.
ANNALS OF MEDICINE
(2023)
Article
Rheumatology
Keina Yomono, Masataka Kuwana
Summary: This study aimed to investigate the benefits of early therapeutic intervention in patients with early SSc. The results showed that the early intervention group had a lower proportion of stable pulmonary function, a higher reduction in active disease, and significantly lower rates of clinical worsening compared to the delayed intervention group.
Article
Rheumatology
Sabrina Hoa, Murray Baron, Marie Hudson
Summary: This international survey reveals regional variations in SSc-ILD screening and significant heterogeneity among rheumatologists and respirologists in the treatment of subclinical SSc-ILD. High-quality research is needed to produce evidence-based guidelines and harmonize the approach to identification and treatment of subclinical SSc-ILD.
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
Biochemistry & Molecular Biology
Veronica Pulito-Cueto, Sara Remuzgo-Martinez, Fernanda Genre, Belen Atienza-Mateo, Victor M. Mora-Cuesta, David Iturbe-Fernandez, Leticia Lera-Gomez, Raquel Perez-Fernandez, Diana Prieto-Pena, Virginia Portilla, Ricardo Blanco, Alfonso Corrales, Oreste Gualillo, Jose M. Cifrian, Raquel Lopez-Mejias, Miguel A. Gonzalez-Gay
Summary: Research has shown a significant increase in EPC frequency in SSc-ILD+ patients, which may be related to the pathogenic processes of vasculopathy and lung fibrosis. Additionally, EPC frequency was higher in male patients and negatively correlated with disease duration in SSc patients.
Article
Cell Biology
Gianluca Bagnato, Antonio Giovanni Versace, Daniela La Rosa, Alberta De Gaetano, Egidio Imbalzano, Marianna Chiappalone, Carmelo Ioppolo, William Neal Roberts, Alessandra Bitto, Natasha Irrera, Alessandro Allegra, Giovanni Pioggia, Sebastiano Gangemi
Summary: Autologous hematopoietic stem cell transplantation has shown improved outcomes in the treatment of severe systemic sclerosis, but there are still challenges such as accurate patient selection and pre-transplant analysis.
Article
Medicine, General & Internal
Cosimo Bruni, Lavinia Mattolini, Lorenzo Tofani, Luna Gargani, Nicholas Landini, Nicola Roma, Gemma Lepri, Martina Orlandi, Serena Guiducci, Silvia Bellando-Randone, Chiara Romei, Yukai Wang, Marco Matucci-Cerinic
Summary: The study found that lung ultrasound (LUS) can be used as a tool to evaluate systemic sclerosis-related interstitial lung disease (SSc-ILD) and can be correlated with different methods of ILD evaluation.
