Article
Respiratory System
Jonathan Keow, Matthew J. Cecchini, Nathashi Jayawardena, Maurizio Zompatori, Mariamma G. Joseph, Marco Mura
Summary: This study found that p16-positive foci can be used to diagnose idiopathic pulmonary fibrosis and are not related to radiographic patterns. High p16 expression is associated with shorter survival time, while antifibrotic therapy has a significant protective effect. Digital spatial profiling showed that fibroblastic foci have distinct gene expression compared to normal and fibrotic areas.
RESPIRATORY RESEARCH
(2022)
Article
Respiratory System
Xiaoping Wu, Yunju Jeong, Sergio Poli de Frias, Imaani Easthausen, Katherine Hoffman, Clara Oromendia, Shahrad Taheri, Anthony J. Esposito, Luisa Quesada Arias, Ehab A. Ayaub, Rie Maurer, Ritu R. Gill, Hiroto Hatabu, Mizuki Nishino, Michelle L. Frits, Christine K. Iannaccone, Michael E. Weinblatt, Nancy A. Shadick, Paul F. Dellaripa, Augustine M. K. Choi, Edy Y. Kim, Ivan O. Rosas, Fernando J. Martinez, Tracy J. Doyle
Summary: The study identified molecular signatures associated with interstitial lung disease in rheumatoid arthritis, providing potential non-invasive diagnostic tools and future therapeutic targets.
Article
Radiology, Nuclear Medicine & Medical Imaging
Ilyes Benlala, Agnes Albat, Elodie Blanchard, Julie Macey, Chantal Raherison, Thomas Benkert, Patrick Berger, Francois Laurent, Gael Dournes
Summary: This study assessed the feasibility of quantifying T-2 interstitial lung disease signal-intensity volume using a semi-automated method and found that T-2-ISIV was significantly correlated with disease severity in IPF patients and higher in IPF patients compared to controls.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2021)
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
Critical Care Medicine
Tsuyoshi Takahashi, Yuriko Terada, Michael K. Pasque, Jingxia Liu, Derek E. Byers, Chad A. Witt, Ruben G. Nava, Varun Puri, Benjamin D. Kozower, Bryan F. Meyers, Daniel Kreisel, G. Alexander Patterson, Ramsey R. Hachem
Summary: CPFE, a characteristic syndrome of smoking-related interstitial lung disease, is associated with higher risks of primary graft dysfunction, acute cellular rejection, and chronic lung allograft dysfunction after lung transplantation. However, the survival rate after transplantation is not significantly different between CPFE and IPF patients.
Review
Cell Biology
Shengnan Yang, Peipei Liu, Yale Jiang, Zai Wang, Huaping Dai, Chen Wang
Summary: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with unknown causes, and currently the only medications that can slow down the progression of the disease and improve survival rate are pirfenidone and nintedanib. The use of mesenchymal stem cells (MSCs) provides a new hope for treating interstitial lung disease, but optimal treatment protocols are still lacking.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Roxana-Elena Cirjaliu, Mariana Deacu, Ioana Gherghisan, Angela Stefania Marghescu, Manuela Enciu, Gabriela Izabela Baltatescu, Antonela Anca Nicolau, Doina-Ecaterina Tofolean, Oana Cristina Arghir, Ariadna-Petronela Fildan
Summary: This review provides a comprehensive analysis of the risk factors, clinical, radiologic, and histological features of both post-COVID-19 pulmonary fibrosis (PCPF) and idiopathic pulmonary fibrosis (IPF). It highlights the similarities and differences between these two diseases by gathering relevant articles published in English up until October 2022 using multiple databases. This review aims to assist clinicians, pathologists, and researchers in making accurate diagnoses and selecting patients for anti-fibrotic therapies and future therapeutic perspectives.
Article
Biochemistry & Molecular Biology
Veronica Pulito-Cueto, Fernanda Genre, Raquel Lopez-Mejias, Victor Manuel Mora-Cuesta, David Iturbe-Fernandez, Virginia Portilla, Maria Sebastian Mora-Gil, Javier Gonzalo Ocejo-Vinyals, Oreste Gualillo, Ricardo Blanco, Alfonso Corrales, Ivan Ferraz-Amaro, Santos Castaneda, Jose Manuel Cifrian Martinez, Belen Atienza-Mateo, Sara Remuzgo-Martinez, Miguel Angel Gonzalez-Gay
Summary: The aim of this study was to investigate the role of endothelin-1 (ET-1) as a biomarker of interstitial lung disease (ILD), and to differentiate between idiopathic pulmonary fibrosis (IPF) and ILD associated with autoimmune diseases (AD-ILD). The study included a large cohort of ILD patients and healthy controls, and found that ET-1 levels were increased in IPF and AD-ILD patients compared to controls. However, ET-1 levels could not differentiate between IPF and AD-ILD, and were associated with worse lung function in IPF and RA-ILD patients.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Medicine, Research & Experimental
Menglin Zou, Xingxing Hu, Weiwei Song, Han Gao, Changrong Wu, Weishuai Zheng, Zhenshun Cheng
Summary: This study found that plasma LTBP2 levels can serve as a biomarker for differentiating CTD-ILD and IPF, and assessing their fibrotic activity. Additionally, clinical evaluation of LTBP2 may be helpful in identifying the presence of the UIP pattern in RA-ILD and discriminating IPF from CTD-UIP, particularly RA-UIP.
CLINICAL AND EXPERIMENTAL MEDICINE
(2023)
Article
Respiratory System
Aparna C. Swaminathan, Anne S. Hellkamp, Megan L. Neely, Shaun Bender, Luca Paoletti, Eric S. White, Scott M. Palmer, Timothy P. M. Whelan, Daniel F. Dilling
Summary: This study identifies the factors associated with lung transplant or death in patients with idiopathic pulmonary fibrosis (IPF). Age, income, and access to lung transplant centers are found to influence the likelihood of receiving a lung transplant or dying. Interventions are needed to address the socioeconomic disparities in lung transplantation.
ANNALS OF THE AMERICAN THORACIC SOCIETY
(2022)
Article
Medicine, General & Internal
Masamichi Komatsu, Hiroshi Yamamoto, Yoshiaki Kitaguchi, Satoshi Kawakami, Mina Matsushita, Takeshi Uehara, Takumi Kinjo, Yosuke Wada, Takashi Ichiyama, Kazuhisa Urushihata, Atsuhito Ushiki, Masanori Yasuo, Masayuki Hanaoka
Summary: Progressive fibrosing interstitial lung disease (PF-ILD) is a type of fibrosing ILD with unclear clinical characteristics and poor prognosis. Patients with PF-ILD require more intensive treatment and have a higher mortality rate, similar to that of patients with idiopathic pulmonary fibrosis.
Review
Biochemistry & Molecular Biology
Sheiphali Gandhi, Roberto Tonelli, Margaret Murray, Anna Valeria Samarelli, Paolo Spagnolo
Summary: Idiopathic pulmonary fibrosis (IPF) is a common and severe disease that primarily affects middle-aged and elderly males. In addition to aging, occupational and environmental exposures, as well as lifestyle factors such as smoking and diet, have been associated with an increased risk of IPF. Trigger factors may also lead to acute exacerbations and poor prognosis.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Medicine, General & Internal
Tinne Goos, Laurens J. De Sadeleer, Jonas Yserbyt, Geert M. Verleden, Marie Vermant, Stijn E. Verleden, Wim A. Wuyts
Summary: A significant portion of ILD patients may develop a progressive fibrosing phenotype similar to IPF, with shared pathophysiological mechanisms and emerging treatment options showing promise for non-IPF ILDs.
JOURNAL OF CLINICAL MEDICINE
(2021)
Article
Respiratory System
Nevins W. Todd, Sergei P. Atamas, Stella E. Hines, Irina G. Luzina, Nirav G. Shah, Edward J. Britt, Andrew J. Ghio, Jeffrey R. Galvin
Summary: The focus of research on idiopathic interstitial pneumonias (IIPs) lies in understanding the association between radiologic and histologic patterns of lung injury and various diseases and environmental factors. The term idiopathic is misleading as these diseases are often associated with diverse etiologies, and a shift towards more etiology-focused nomenclature is beneficial for better management and understanding of interstitial lung diseases (ILDs).
EXPERT REVIEW OF RESPIRATORY MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Filippo Patrucco, Carlo Albera, Mattia Bellan, Martina Zava, Francesco Gavelli, Piero Emilio Balbo, Paolo Solidoro
Summary: This study evaluated the reliability of ReDS (TM) technology in patients with idiopathic pulmonary fibrosis (IPF) and healthy volunteers, and investigated the correlation between ReDS (TM) score and clinical, radiological, and functional parameters. The results showed a higher correlation between ReDS (TM) score and lung function in IPF patients, with no correlation observed in the healthy control group.
RESPIRATORY MEDICINE
(2023)
Article
Critical Care Medicine
Laurens J. De Sadeleer, John E. McDonough, Jonas C. Schupp, Xiting Yan, Arno Vanstapel, Anke Van Herck, Stephanie Everaerts, Vincent Geudens, Annelore Sacreas, Tinne Goos, Celine Aelbrecht, Tim S. Nawrot, Dries S. Martens, Dominique Schols, Sandra Claes, Johny A. Verschakelen, Eric K. Verbeken, Maximilian Ackermann, Anabelle Decottignies, Manon Mahieu, Tillie-Louise Hackett, James C. Hogg, Bart M. Vanaudenaerde, Stijn E. Verleden, Naftali Kaminski, Wim A. Wuyts
Summary: This study identified molecular traits that characterize the morphological progression of fibrotic hypersensitivity pneumonitis (fHP) and their associations with clinical behavior. Comparing with idiopathic pulmonary fibrosis (IPF), the transcriptome landscape is determined by local disease extent rather than diagnosis alone.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Article
Critical Care Medicine
Laurens J. De Sadeleer, Stijn E. Verleden, Jonas C. Schupp, John E. McDonough, Tinne Goos, Jonas Yserbyt, Elena Bargagli, Paola Rottoli, Naftali Kaminski, Antje Prasse, Wim A. Wuyts
Summary: Gene expression-based endotyping is feasible in IPF and can inform clinical evolution, opening up the possibility of personalized therapy.
Article
Multidisciplinary Sciences
Maor Sauler, John E. McDonough, Taylor S. Adams, Neeharika Kothapalli, Thomas Barnthaler, Rhiannon B. Werder, Jonas C. Schupp, Jessica Nouws, Matthew J. Robertson, Cristian Coarfa, Tao Yang, Maurizio Chioccioli, Norihito Omote, Carlos Cosme, Sergio Poli, Ehab A. Ayaub, Sarah G. Chu, Klaus H. Jensen, Jose L. Gomez, Clemente J. Britto, Micha Sam B. Raredon, Laura E. Niklason, Andrew A. Wilson, Pascal N. Timshel, Naftali Kaminski, Ivan O. Rosas
Summary: Chronic obstructive pulmonary disease (COPD) is a leading cause of death worldwide, but our understanding of cell-specific mechanisms underlying COPD pathobiology remains incomplete. In this study, the authors used single-cell RNA sequencing to analyze human lung tissue and identified transcriptional changes in alveolar niche cells associated with the disease.
NATURE COMMUNICATIONS
(2022)
Article
Multidisciplinary Sciences
Avraham Unterman, Tomokazu S. Sumida, Nima Nouri, Xiting Yan, Amy Y. Zhao, Victor Gasque, Jonas C. Schupp, Hiromitsu Asashima, Yunqing Liu, Carlos Cosme, Wenxuan Deng, Ming Chen, Micha Sam Brickman Raredon, Kenneth B. Hoehn, Guilin Wang, Zuoheng Wang, Giuseppe DeIuliis, Neal G. Ravindra, Ningshan Li, Christopher Castaldi, Patrick Wong, John Fournier, Santos Bermejo, Lokesh Sharma, Arnau Casanovas-Massana, Chantal B. F. Vogels, Anne L. Wyllie, Nathan D. Grubaugh, Anthony Melillo, Hailong Meng, Yan Stein, Maksym Minasyan, Subhasis Mohanty, William E. Ruff, Inessa Cohen, Khadir Raddassi, Laura E. Niklason, Albert Ko, Ruth R. Montgomery, Shelli F. Farhadian, Akiko Iwasaki, Albert C. Shaw, David van Dijk, Hongyu Zhao, Steven H. Kleinstein, David A. Hafler, Naftali Kaminski, Charles S. Dela Cruz
Summary: Using multi-omics single-cell analysis, this study reveals the immune abnormalities associated with progressive COVID-19, including S100A(hi)/HLA-DRlo classical monocytes and activated LAG-3(hi) T cells. It also finds a correlation between T cell receptor repertoire and B cell somatic hypermutation frequency with disease progression.
NATURE COMMUNICATIONS
(2022)
Article
Pathology
Jan C. Kamp, Lavinia Neubert, Maximilian Ackermann, Helge Stark, Edith Plucinski, Harshit R. Shah, Sabina Janciauskiene, Anke K. Bergmann, Gunnar Schmidt, Tobias Welte, Axel Haverich, Christopher Werlein, Peter Braubach, Florian Laenger, Nicolaus Schwerk, Karen M. Olsson, Jan Fuge, Da-Hee Park, Jonas C. Schupp, Marius M. Hoeper, Mark P. Kuehnel, Danny D. Jonigk
Summary: This study analyzed the microvascular morphology and molecular background of ACD, revealing characteristics such as altered capillary network, high prevalence of intussusceptive angiogenesis, and increased activity of key signaling pathways in ACD patients.
AMERICAN JOURNAL OF PATHOLOGY
(2022)
Article
Cell Biology
Stefan Preisendoerfer, Yoshihiro Ishikawa, Elisabeth Hennen, Stephan Winklmeier, Jonas C. Schupp, Larissa Knueppel, Isis E. Fernandez, Leonhard Binzenhofer, Andrew Flatley, Brenda M. Juan-Guardela, Clemens Ruppert, Andreas Guenther, Marion Frankenberger, Rudolf A. Hatz, Nikolaus Kneidinger, Jurgen Behr, Regina Feederle, Aloys Schepers, Anne Hilgendorff, Naftali Kaminski, Edgar Meinl, Hans Peter Baechinger, Oliver Eickelberg, Claudia A. Staab-Weijnitz
Summary: The study reveals that FKBP11 plays an important role in plasma cells, is regulated by XBP1, and sheds light on the important mechanisms involved in antibody production and folding.
Article
Critical Care Medicine
Farida Ahangari, Christine Becker, Daniel G. Foster, Maurizio Chioccioli, Meghan Nelson, Keriann Beke, Xing Wang, Aurelien Justet, Taylor Adams, Benjamin Readhead, Carly Meador, Kelly Correll, Loukia N. Lili, Helen M. Roybal, Kadi-Ann Rose, Shuizi Ding, Thomas Barnthaler, Natalie Briones, Giuseppe DeIuliis, Jonas C. Schupp, Qin Li, Norihito Omote, Yael Aschner, Lokesh Sharma, Katrina W. Kopf, Bjorn Magnusson, Ryan Hicks, Anna Backmark, Charles S. Dela Cruz, Ivan Rosas, Leslie P. Cousens, Joel T. Dudley, Naftali Kaminski, Gregory P. Downey
Summary: This study identifies the potential therapeutic effectiveness of saracatinib, a Src kinase inhibitor, in treating pulmonary fibrosis. The research shows that saracatinib is as effective as or even superior to currently approved antifibrotic drugs in blocking fibrogenic responses. Transcriptomic analysis reveals specific gene sets associated with fibrosis that are uniquely altered by saracatinib.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2022)
Article
Multidisciplinary Sciences
Benedikt Jaeger, Jonas Christian Schupp, Linda Plappert, Oliver Terwolbeck, Gian Kayser, Peggy Engelhard, Taylor Sterling Adams, Robert Zweigerdt, Henning Kempf, Stefan Lienenklaus, Wiebke Garrels, Irina Nazarenko, Danny Jonigk, Malgorzata Wygrecka, Denise Klatt, Axel Schambach, Naftali Kaminski, Antje Prasse, Nataliia Artysh
Summary: This study focuses on the properties of airway basal cells (ABC) obtained from patients with idiopathic pulmonary fibrosis (IPF) and reveals the extensive reprogramming of IPF-ABC. The study also shows that IPF-ABC can induce fibroblast proliferation and extracellular matrix deposition in a 3D organoid model, and these changes can be reversed by SRC inhibition. The findings suggest that IPF-ABC and SRC inhibition may serve as potential targets for the treatment of IPF.
NATURE COMMUNICATIONS
(2022)
Article
Biochemical Research Methods
Ye Yuan, Carlos Cosme, Taylor Sterling Adams, Jonas Schupp, Koji Sakamoto, Nikos Xylourgidis, Matthew Ruffalo, Jiachen Li, Naftali Kaminski, Ziv Bar-Joseph
Summary: Studies comparing single cell RNA-Seq data mainly focus on differences in cell types and differentially expressed genes. However, inferring cell-cell interactions without spatial information is challenging. To overcome this, researchers developed a pipeline called CINS, which combines Bayesian network analysis and regression-based modeling to identify differential cell type interactions and associated proteins between conditions.
PLOS COMPUTATIONAL BIOLOGY
(2022)
Article
Cell Biology
Hasan H. Oz, Ee-Chun Cheng, Caterina Di Pietro, Toma Tebaldi, Giulia Biancon, Caroline Zeiss, Ping-Xia Zhang, Pamela H. Huang, Sofia S. Esquibies, Clemente J. Britto, Jonas C. Schupp, Thomas S. Murray, Stephanie Halene, Diane S. Krause, Marie E. Egan, Emanuela M. Bruscia
Summary: This study identifies CCR2+ monocytes as a previously neglected contributor to lung damage in CF, driving pathogenic TGF-β signaling and sustaining inflammation by facilitating neutrophil recruitment. Targeting CCR2 to reduce monocyte numbers in CF lungs can ameliorate neutrophil inflammation and prevent tissue damage.
Article
Medicine, Research & Experimental
Farida Ahangari, Nathan L. Price, Shipra Malik, Maurizio Chioccioli, Thomas Barnthaler, Taylor S. Adams, Jooyoung Kim, Sai Pallavi Pradeep, Shuizi Ding, Carlos Cosmos Jr., Kadi-Ann S. Rose, John E. McDonough, Nachelle R. Aurelien, Gabriel Ibarra, Norihito Omote, Jonas C. Schupp, Giuseppe DeIuliis, Julian A. Villalba Nunez, Lokesh Sharma, Changwan Ryu, Charles S. Dela Cruz, Xinran Liu, Antje Prasse, Ivan Rosas, Raman Bahal, Carlos Fernandez-Hernando, Naftali Kaminski
Article
Critical Care Medicine
Toyoshi Yanagihara, Kazuya Tsubouchi, Quan Zhou, Michael Chong, Kohei Otsubo, Takuma Isshiki, Jonas C. Schupp, Seidai Sato, Ciaran Scallan, Chandak Upagupta, Spencer Revill, Anmar Ayoub, Sy Giin Chong, Anna Dvorkin-Gheva, Naftali Kaminski, Jussi Tikkanen, Shaf Keshavjee, Guillaume Pare, Christophe Guignabert, Kjetil Ask, Martin R. J. Kolb
Summary: This study investigated the role of vascular remodeling in fibrotic lungs and its contribution to the progression of fibrosis. It was found that endothelial dysfunction and activation of vascular smooth muscle cells enhance fibrogenesis through impaired BMPR2 signaling. Tacrolimus may have therapeutic potential in treating advanced IPF and concomitant PH. Additionally, genetic abnormalities may determine the development of PH in advanced IPF.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Editorial Material
Critical Care Medicine
Jonas C. Schupp, Naftali Kaminski
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Letter
Critical Care Medicine
Jonas C. Schupp, Edward P. Manning, Maurizio Chioccioli, Jan C. Kamp, Leonard Christian, Changwan Ryu, Erica Herzog, Mark P. Kuehnel, Antje Prasse, Naftali Kaminski, Danny D. Jonigk, Robert J. Homer
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Medicine, General & Internal
Sandra Freitag-Wolf, Jonas C. Schupp, Bjoern C. Frye, Annegret Fischer, Raihanatul Anwar, Robert Kieszko, Violeta Mihailovic-Vucinic, Janusz Milanowski, Dragana Jovanovic, Gernot Zissel, Elena Bargagli, Paola Rottoli, Dragos Bumbacea, Rene Jonkers, Ling-Pei Ho, Karoline I. Gaede, Anna Dubaniewicz, Ben G. Marshall, Andreas Guenther, Martin Petrek, Michael P. Keane, Sigridur O. Haraldsdottir, Francesco Bonella, Christian Grah, Tatjana Peros-Golubicic, Zamir Kadija, Stefan Pabst, Christian Grohe, Janos Strausz, Martina Safrankova, Ann Millar, Jiri Homolka, Wim A. Wuyts, Lisa G. Spencer, Michael Pfeifer, Dominique Valeyre, Venerino Poletti, Hubertus Wirtz, Antje Prasse, Stefan Schreiber, Astrid Dempfle, Joachim Mueller-Quernheim
Summary: This study investigated the association of genetic markers with different phenotypes using meta-analysis. The results showed no significant genetic association in the overall cohort, but confirmed the association of acute onset with TNF and reported a new association with HLA polymorphism in the Serbian sub-cohort. The study also suggested that gene-environment interactions may influence the clinical phenotype and different geographic regions may have distinct genetic links and environmental factors.
FRONTIERS IN MEDICINE
(2023)
