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.
Article
Medicine, General & Internal
Liticia Chikhoune, Thierry Brousseau, Sandrine Morell-Dubois, Meryem Maud Farhat, Helene Maillard, Emmanuel Ledoult, Marc Lambert, Cecile Yelnik, Sebastien Sanges, Vincent Sobanski, Eric Hachulla, David Launay
Summary: This study aimed to assess the association of routine laboratory parameters with the severity and progression of systemic sclerosis (SSc). The results showed that certain routine laboratory parameters were associated with different clinical presentations and outcomes of SSc.
JOURNAL OF CLINICAL MEDICINE
(2022)
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
Medicine, Research & Experimental
Masataka Kuwana, Aiko Saito, Wataru Sakamoto, Christina Raabe, Kumiko Saito
Summary: This study estimated the incidence and prevalence rates of systemic sclerosis (SSc) and SSc-associated interstitial lung disease (SSc-ILD) in Japan using medical databases. The results showed that approximately 30% of SSc patients had ILD, and the utilization of immunomodulatory medications was low.
ADVANCES IN THERAPY
(2022)
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
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
Medicine, General & Internal
Ai Kuzumi, Ayumi Yoshizaki, Satoshi Ebata, Takemichi Fukasawa, Asako Yoshizaki-Ogawa, Yoshihide Asano, Koji Oba, Shinichi Sato
Summary: The study found that serum TARC levels were elevated in SSc patients, especially in those with the diffuse subtype and SSc-associated ILD. However, there was no significant correlation between serum TARC levels with pulmonary function and serum levels of interleukin-13. Longitudinal study showed that serum TARC levels did not predict the onset or progression of SSc-ILD in patients with SSc.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Cardiac & Cardiovascular Systems
Raphael Hurtubise, Marie Hudson, Genevieve Gyger, Mianbo Wang, Russell J. Steele, Murray Baron, Sabrina Hoa
Summary: In this large retrospective cohort study, the use of gastroprotective or promotility agents was not associated with a reduced risk of development of clinically apparent SSc-ILD.
RESPIRATORY MEDICINE
(2021)
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
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
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
Critical Care Medicine
Richard J. Allen, Justin M. Oldham, David A. Jenkins, Olivia C. Leavy, Beatriz Guillen-Guio, Carl A. Melbourne, Shwu-Fan Ma, Jonathan Jou, John S. Kim, William A. Fahy, Eunice Oballa, Richard B. Hubbard, Vidya Navaratnam, Rebecca Braybrooke, Gauri Saini, Katy M. Roach, Martin D. Tobin, Nik Hirani, Moira K. B. Whyte, Naftali Kaminski, Yingze Zhang, Fernando J. Martinez, Angela L. Linderholm, Ayodeji Adegunsoye, Mary E. Strek, Toby M. Maher, Philip L. Molyneaux, Carlos Flores, Imre Noth, R. Gisli Jenkins, Louise Wain
Summary: In this study, a genetic variant associated with disease progression in idiopathic pulmonary fibrosis (IPF) was identified, and the PKN2 gene was proposed as a potential target for novel therapeutic approaches. This finding provides insights into the biological mechanisms underlying IPF and offers potential implications for its treatment.
LANCET RESPIRATORY MEDICINE
(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
Respiratory System
Anais Roeser, Lucile Sese, Guillaume Chassagnon, Benjamin Chaigne, Bertrand Dunogue, Stephane Tran Ba, Salma Jebri, Pierre-Yves Brillet, Marie Pierre Revel, Frederique Aubourg, Robin Dhote, Frederic Caux, Isabella Annesi-Maesano, Luc Mouthon, Hilario Nunes, Yurdaguel Uzunhan
Summary: This study investigated the association between air pollution exposure and the severity and progression of interstitial lung disease (ILD) in patients with systemic sclerosis (SSc)-associated ILD. The results showed that exposure to ozone (O-3) was associated with more severe ILD at diagnosis and progression at 24 months.
RESPIRATORY RESEARCH
(2023)
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.
