Article
Radiology, Nuclear Medicine & Medical Imaging
Joanna G. Escalon, Alan C. Legasto, Dennis Toy, James F. Gruden
Summary: In this study, central paradiaphragmatic middle lobe (ML) involvement was found in most NSIP patients, with high interobserver agreement. Fibrosis was present in nearly all cases, while other features such as subpleural sparing, homogeneity, and bronchiectasis varied among patients.
EUROPEAN RADIOLOGY
(2021)
Article
Respiratory System
Noriyuki Enomoto, Sakae Homma, Naohiko Inase, Yasuhiro Kondoh, Takeshi Saraya, Hajime Takizawa, Yoshikazu Inoue, Hiroshi Ishii, Yoshio Taguchi, Shinyu Izumi, Yasuhiko Yamano, Yoshinori Tanino, Yasuhiko Nishioka, Mikio Toyoshima, Koshi Yokomura, Shiro Imokawa, Naoki Koshimizu, Takehisa Sano, Taisuke Akamatsu, Hiroshi Mukae, Motoyasu Kato, Naoki Hamada, Hirofumi Chiba, Shinobu Akagawa, Shigeo Muro, Hironori Uruga, Hiroyuki Matsuda, Yusuke Kaida, Miho Kanai, Kazutaka Mori, Masafumi Masuda, Hironao Hozumi, Tomoyuki Fujisawa, Yutaro Nakamura, Noriyoshi Ogawa, Takafumi Suda
Summary: This nationwide multicentre study prospectively evaluated 376 patients with IIP, finding that 18.6% of them met the IPAF criteria. Patients with IPAF were more likely to develop systemic autoimmune diseases, had fewer acute exacerbations of IIP, and showed better survival outcomes. Cluster analysis identified a subgroup with more autoimmune features, predominantly female, and with a better prognosis.
Article
Medicine, General & Internal
Jonathan H. Chung, Ayodeji Adegunsoye, Brenna Cannon, Rekha Vij, Justin M. Oldham, Christopher King, Steven M. Montner, Prahasit Thirkateh, Scott Barnett, Ronald Karwoski, Brian J. Bartholmai, Mary Strek, Steven D. Nathan
Summary: A study comparing quantitative imaging data between IPF and CTD-ILD in a UIP setting found that VRS was higher in IPF patients. VRS showed an inverse correlation with DLCO%, while the total volume of normal lung was associated with both DLCO% and FVC% in multivariable analysis.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Medical Laboratory Technology
Qipan Zhang, Lun Guo, Xuxiang Song, Chengna Lv, Pan Tang, Yan Li, Qunli Ding, Mingcai Li
Summary: The study revealed significantly higher levels of IL-36 alpha and IL-36 gamma in CTD-ILD and IPF patients compared to healthy controls, while IL-36Ra levels did not differ significantly. Increased IL-36 levels were associated with disease severity in IPF patients, with high diagnostic value demonstrated by ROC curve analysis for IL-36 alpha in both IPF and CTD-ILD patients.
CLINICA CHIMICA ACTA
(2022)
Article
Rheumatology
G. Sambataro, C. A. Ferrara, S. E. Torrisi, C. Spadaro, G. Vignigni, A. Vancheri, N. Del Papa, M. Orlandi, M. Colaci, L. Malatino, S. Palmucci, L. Cavagna, D. Sambataro, C. Vancheri
Summary: The study aims to evaluate the rate of progression towards SAD in UIP patients satisfying only one IPAF domain compared to classic IPF, and found that 28.9% of UIPAF patients progressed towards SAD.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2022)
Article
Immunology
Dongdong Li, Liting Ding, Jiao Luo, Qiu-Gen Li
Summary: The study aimed to construct a nomogram using machine learning to predict the 90-day mortality risk in pneumonia patients with CTD treated with glucocorticoids or/and immunosuppressants. The key variables for predicting the mortality risk were fever, cyanosis, blood urea nitrogen, ganciclovir treatment, and anti-pseudomonas treatment. The nomogram showed good predictive performance in both the training and validation cohorts.
FRONTIERS IN IMMUNOLOGY
(2023)
Review
Rheumatology
Eshwar Umashankar, Christina Abdel-Shaheed, Marshall Plit, Laila Girgis
Summary: This study conducted a meta-analysis on the efficacy of nailfold videocapillaroscopy (NVC) in subgroups of connective tissue disease-related interstitial lung disease (CTD-ILD), interstitial pneumonia with autoimmune features (IPAF), and idiopathic pulmonary fibrosis (IPF). The results showed that NVC can increase the diagnostic accuracy of ILD when used in a multi-disciplinary setting, with the highest utility in CTD-ILD, followed by IPAF and IPF. The late scleroderma pattern was found to be the most frequent nailfold capillary pattern.
Article
Biochemistry & Molecular Biology
Takuya Kotani, Masaki Ikemoto, Shogo Matsuda, Ryota Masutani, Tohru Takeuchi
Summary: In this study, it was found that the hybrid protein hMIKO-1 can suppress the abnormal activation of macrophages, reduce lung fibrosis in mice, and decrease the expression of related inflammation factors, indicating its potential as a therapeutic candidate for ILD treatment.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Pharmacology & Pharmacy
Sebastiano Emanuele Torrisi, Nicolas Kahn, Julia Waelscher, Markus Polke, Joyce S. Lee, Philip L. Molyneaux, Francesca Maria Sambataro, Claus Peter Heussel, Carlo Vancheri, Michael Kreuter
Summary: Almost one-third of fibrosing ILD (fILDs) have a clinical disease behavior similar to IPF, but there are no globally accepted criteria on the definition of a progressive phenotype in non-IPF fILD. Different definitions identify different groups of patients with progressive fILD, which may have important prognostic and therapeutic implications.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Rheumatology
Goncalo Boleto, Silje Reiseter, Anna-Maria Hoffmann-Vold, Adrien Mirouse, Patrice Cacoub, Marco Matucci-Cerinic, Manuel Silverio-Antonio, Joao Eurico Fonseca, Ana Catarina Duarte, Jorge Pestana Lopes, Valeria Riccieri, Alain Lescoat, Erwan Le Tallec, Ivan Castellvi Barranco, Jose Luis Tandaipan, Paolo Airo, Masataka Kuwana, Hoda Kavosi, Jerome Avouac, Yannick Allanore
Summary: This study compared predictive factors for interstitial lung disease (ILD) in patients with mixed connective tissue disease (MTCD) and found different factors associated with ILD. The study also found that MCTD-ILD patients have increased mortality and that digital ulcers (DU) are associated with progressive lung disease.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2023)
Article
Surgery
Amelie Riviere, Clement Picard, Cristina Berastegui, Victor Manuel Mora, Vincent Bunel, Laurent Godinas, Elena Salvaterra, Valeria Rossetti, Laurent Savale, Dominique Israel-Biet, Xavier Demant, Julien Bermudez, Federica Meloni, Peter Jaksch, Jesper Magnusson, Laurence Beaumont, Michael Perch, Jean-Francois Mornex, Christiane Knoop, John-David Aubert, Baptiste Hervier, Hilario Nunes, Marc Humbert, Jens Gottlieb, Yurdagul Uzunhan, Jerome Le Pavec
Summary: This study aimed to evaluate the survival rate and prognostic factors in lung transplant recipients with interstitial lung disease complicating idiopathic inflammatory myopathy (IIM-ILD). The study found that the post-transplant survival rate in IIM-ILD was similar to that in international all-cause transplantation registries and worse in patients with classical IIM. Muscle involvement was identified as the main factor associated with worse survival.
AMERICAN JOURNAL OF TRANSPLANTATION
(2022)
Article
Cardiac & Cardiovascular Systems
Jongmin Lee, Kyuhwan Kim, Yong Suk Jo
Summary: This study compared the prevalence and clinical characteristics of progressive pulmonary fibrosis (PPF) in patients with connective tissue disease (CTD) related interstitial lung disease (ILD) according to different diagnostic criteria. The results showed that the prevalence of PPF varied depending on the diagnostic criteria used, but there was no difference in clinical characteristics.
RESPIRATORY MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Marco Sebastiani, Paola Faverio, Andreina Manfredi, Giulia Cassone, Caterina Vacchi, Anna Stainer, Maria Rosa Pozzi, Carlo Salvarani, Alberto Pesci, Fabrizio Luppi
Summary: Since 2015, there has been significant attention on research related to IPAF, including studies on prevalence, clinical features, pathological features, and prognosis. Recent studies have found that about 15% of IPAF patients may progress to a defined connective tissue disease, and compared to idiopathic pulmonary fibrosis, IPAF patients show better performance in pulmonary function tests and oxygen delivery requirements. Further prospective studies, potentially derived from multicenter cohorts and randomized control trials, are needed to validate the proposed classification criteria.
Article
Medicine, Research & Experimental
Ju Kwang Lee, Yura Ahn, Han Na Noh, Sang Min Lee, Bin Yoo, Chang-Keun Lee, Yong-Gil Kim, Seokchan Hong, Soo Min Ahn, Ho Cheol Kim
Summary: This study reviewed 197 patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) and found that progressive pulmonary fibrosis (PPF) was significantly associated with mortality, as well as increased rates of respiratory-related hospitalizations, pneumonia, acute exacerbations, and weight loss.
CLINICAL AND EXPERIMENTAL MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Giuliana Cerro Chiang, Tanyalak Parimon
Summary: CTD-ILD is a group of systemic autoimmune disorders that cause lung interstitial abnormalities or lung fibrosis. The pathogenesis of CTD-ILD is not well understood, but it shares common risk factors with idiopathic pulmonary fibrosis. The primary mechanism involves dysfunction of alveolar type II cells, triggering inflammatory cascades and leading to abnormal lung remodeling and fibrosis. Understanding these mechanisms is important for developing targeted therapies and identifying disease biomarkers.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Rheumatology
Michael Kreuter, Anna-Maria Hoffmann-Vold, Marco Matucci-Cerinic, Lesley Ann Saketkoo, Kristin B. Highland, Hilary Wilson, Margarida Alves, Elvira Erhardt, Nils Schoof, Toby M. Maher
Summary: This study found that the severity of SSc-ILD and significant deteriorations in lung function are associated with a major impact on health-related quality of life (HRQoL). Treatments that slow lung function decline and prevent severe SSc-ILD are important for preserving HRQoL.
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)
Article
Critical Care Medicine
Anna L. Peljto, Rachel Z. Blumhagen, Avram D. Walts, Jonathan Cardwell, Julia Powers, Tamera J. Corte, Joanne L. Dickinson, Ian Glaspole, Yuben P. Moodley, Martina Koziar Vasakova, Elisabeth Bendstrup, Jesper R. Davidsen, Raphael Borie, Bruno Crestani, Philippe Dieude, Francesco Bonella, Ulrich Costabel, Gunnar Gudmundsson, Seamas C. Donnelly, Jim Egan, Michael T. Henry, Michael P. Keane, Marcus P. Kennedy, Cormac McCarthy, Aoife N. McElroy, Joshua A. Olaniyi, Katherine M. A. O'Reilly, Luca Richeldi, Paolo M. Leone, Venerino Poletti, Francesco Puppo, Sara Tomassetti, Valentina Luzzi, Nurdan Kokturk, Nesrin Mogulkoc, Christine A. Fiddler, Nikhil Hirani, R. Gisli Jenkins, Toby M. Maher, Philip L. Molyneaux, Helen Parfrey, Rebecca Braybrooke, Timothy S. Blackwell, Peter D. Jackson, Steven D. Nathan, Mary K. Porteous, Kevin K. Brown, Jason D. Christie, Harold R. Collard, Oliver Eickelberg, Elena E. Foster, Kevin F. Gibson, Marilyn Glassberg, Daniel J. Kass, Jonathan A. Kropski, David Lederer, Angela L. Linderholm, Jim Loyd, Susan K. Mathai, Sydney B. Montesi, Imre Noth, Justin M. Oldham, Amy J. Palmisciano, Cristina A. Reichner, Mauricio Rojas, Jesse Roman, Neil Schluger, Barry S. Shea, Jeffrey J. Swigris, Paul J. Wolters, Yingze Zhang, Cecilia M. A. Prele, Juan I. Enghelmayer, Maria Otaola, Christopher J. Ryerson, Mauricio Salinas, Martina Sterclova, Tewodros H. Gebremariam, Marjukka Myllarniemi, Roberto G. Carbone, Haruhiko Furusawa, Masaki Hirose, Yoshikazu Inoue, Yasunari Miyazaki, Ken Ohta, Shin Ohta, Tsukasa Okamoto, Dong Soon Kim, Annie Pardo, Moises Selman, Alvaro U. Aranda, Moo Suk Park, Jong Sun Park, Jin Woo Song, Maria Molina-Molina, Lurdes Planas-Cerezales, Gunilla Westergren-Thorsson, Albert V. Smith, Ani W. Manichaikul, John S. Kim, Stephen S. Rich, Elizabeth C. Oelsner, R. Graham Barr, Jerome I. Rotter, Josee Dupuis, George O'Connor, Ramachandran S. Vasan, Michael H. Cho, Edwin K. Silverman, Marvin I. Schwarz, Mark P. Steele, Joyce S. Lee, Ivana V. Yang, Tasha E. Fingerlin, David A. Schwartz
Summary: This study investigated the role of rare variants, genome-wide, on the risk of idiopathic pulmonary fibrosis (IPF) using whole-genome sequencing. The results showed that rare variants within the TERT and RTEL1 genes were significantly associated with IPF, along with well-established common variants. Therefore, including TERT, RTEL1, common variants, and environmental risk factors in risk profiling and IPF therapy development is likely to have the largest impact on this complex disease.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Medicine, General & Internal
Toby M. Maher, Paul Ford, Kevin K. Brown, Ulrich Costabel, Vincent Cottin, Sonye K. Danoff, Irene Groenveld, Eric Helmer, R. Gisli Jenkins, Julie Milner, Geert Molenberghs, Bjorn Penninckx, Matthew J. Randall, Bernt Van den Blink, Ann Fieuw, Charlotte Vandenrijn, Sanda Rocak, Ineke Seghers, Lixin Shao, Amit Taneja, Garrit Jentsch, Timothy R. Watkins, Wim A. Wuyts, Michael Kreuter, Nadia Verbruggen, Niyati Prasad, Marlies S. Wijsenbeek
Summary: This study aimed to assess the efficacy and safety of the autotaxin inhibitor ziritaxestat in patients with idiopathic pulmonary fibrosis (IPF). The study found that ziritaxestat did not improve clinical outcomes compared with placebo in patients with IPF.
JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION
(2023)
Editorial Material
Respiratory System
Elisabetta Renzoni
Editorial Material
Respiratory System
Elisabetta Renzoni, Laura Fabbri
Article
Respiratory System
Claire M. Nolan, Susie J. Schofield, Matthew Maddocks, Suhani Patel, Ruth E. Barker, Jessica A. Walsh, Oliver Polgar, Peter M. George, Philip L. Molyneaux, Toby M. Maher, Paul Cullinan, William D. -C. Man
Summary: This study aimed to investigate the longitudinal change of four-metre gait speed (4MGS) in newly diagnosed individuals with idiopathic pulmonary fibrosis (IPF) and its association with adverse outcome. The study found that 4MGS declined over 6 months, and a decline of 0.07 m/s or more was significantly associated with increased risk of hospitalization/death. Therefore, 4MGS change has the potential to serve as a surrogate endpoint for interventions targeting hospitalization/death risk.
Editorial Material
Critical Care Medicine
Felix Chua, Suying Low, Gin Tsen Chai, Yoshikazu Inoue, Voon Ong, Amornpun Wongkarnjana, Kamon Kawkitinarong, Jin Woo Song, Zuhanis Abdul Hamid, Aida Abdul Aziz, Mae Campomanes, Toby M. Maher, Philip L. Molyneaux, Syazatul Syakirin
LANCET RESPIRATORY MEDICINE
(2023)
Letter
Respiratory System
Justin M. Oldham, Kirk W. Johnson, Gesa J. Albers, Emily Calamita, Jordina Mah, Poonam Ghai, Richard J. Hewitt, Toby M. Maher, Philip L. Molyneaux, Michael Huang, Adam J. Byrne
Article
Respiratory System
Steve Jones, Maxine Flewett, Ron Flewett, Sharon Lee, Bill Vick, Milla Thompson, Sabine Pinnetti, Donald F. Zoz, Anna-Maria Hoffmann-Vold, Michael Kreuter, Toby M. Maher
Summary: This study aimed to understand and address the barriers associated with trial participation for patients with pulmonary fibrosis. Virtual simulations were conducted and participants' feedback led to patient-friendly adaptations being made to the trial protocol and conduct, resulting in improved recruitment and retention rates and ultimately, improved data quality.
Article
Respiratory System
Toby M. Maher, Susanne Stowasser, Florian Voss, Elisabeth Bendstrup, Michael Kreuter, Fernando J. Martinez, Patricia J. Sime, Christian Stock
Summary: This study used clinical trial data of nintedanib in patients with pulmonary fibrosis to assess decline in forced vital capacity (FVC) as a surrogate for mortality. The results showed a strong association between the rate of decline in FVC % predicted and the risk of death over 52 weeks, supporting FVC decline as a surrogate for mortality in these patients.
Article
Respiratory System
Carmel J. W. Stock, William G. Bray, Vasilis Kouranos, Joseph Jacob, Maria Kokosi, Peter M. George, Felix Chua, Athol U. Wells, Piersante Sestini, Elisabetta A. Renzoni
Summary: This study found that higher baseline serum CRP levels are independently associated with reduced 5-year survival in four different ILDs, including IPF, fHP, RA-ILD, and SSc-ILD.
Article
Multidisciplinary Sciences
Richard J. Hewitt, Franz Puttur, David C. A. Gaboriau, Frederic Fercoq, Maryline Fresquet, William J. Traves, Laura L. Yates, Simone A. Walker, Philip L. Molyneaux, Samuel V. Kemp, Andrew G. Nicholson, Alexandra Rice, Edward Roberts, Rachel Lennon, Leo M. Carlin, Adam J. Byrne, Toby M. Maher, Clare M. Lloyd
Summary: This study reveals the impact of changes in extracellular matrix (ECM) composition secreted by lung fibroblasts on the migration and function of KRT5(+) cells in idiopathic pulmonary fibrosis (IPF), contributing to tissue remodeling in the fibrotic lung.
NATURE COMMUNICATIONS
(2023)
Article
Respiratory System
Peter Saunders, Zhe Wu, William A. Fahy, Iain D. Stewart, Gauri Saini, David J. F. Smith, Rebecca Braybrooke, Carmel Stock, Elisabetta A. Renzoni, Simon R. Johnson, R. Gisli Jenkins, Maria G. Belvisi, Jaclyn A. Smith, Toby M. Maher, Philip L. Molyneaux
Summary: This study aims to assess the burden of cough and its impact on quality of life in newly diagnosed IPF patients. The results indicate that cough burden in IPF is high, but cough-specific quality of life does not have prognostic value and is not associated with MUC5B promoter polymorphism.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2023)
Meeting Abstract
Respiratory System
Toby M. Maher, Thomas Sciascia, Enoch Bortey, Philip L. Molyneaux, Marlies Wijsenbeek, Jaclyn Smith
