Serum metalloproteinase-7 as a biomarker of progressive pulmonary fibrosis by Márcia Araújo, Marília Beltrão, Oksana Sokhatska, Natália Melo, Patrícia Caetano Mota, Helder Novais Bastos, André Terras, David Coelho, Luís Delgado, António Morais

“…Introduction Progressive pulmonary fibrosis (PPF) corresponds to any fibrotic interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF) that presents clinical, physiological and/or radiological evidence of disease progression similar to IPF. …”
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Features and predictive value of 6-min walk test outcomes in interstitial lung disease: an observation study using wearable monitors by Jiaying Li, Xiaobing Wu, Xiaoyan Li, Miaozhen Deng, Xinyin Liang, Huiqun Wei

“…Objectives To describe 6-min walk test (6MWT) outcomes, and to investigate their correlations with cardiopulmonary and lung function among patients with interstitial lung disease (ILD) which was not limited to idiopathic pulmonary fibrosis.Methods We collected patients’ demographic data and obtained minute-by-minute 6MWT outcomes. …”
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European ILD registry algorithm for self-assessment in interstitial lung diseases (eurILDreg ASA-ILD). by Ekaterina Krauss, Laurenz H Claas, Silke Tello, Jennifer Naumann, Sandra Wobisch, Stefan Kuhn, Raphael W Majeed, Karen Moor, Maria Molina-Molina, Oisin Byrne, Rebecca Borton, Marlies S Wijsenbeek, Nik Hirani, Carlo Vancheri, Bruno Crestani, Andreas Guenther, eurILDreg investigators

“…<h4>Background and aims</h4>Predicting progression and prognosis in Interstitial Lung Diseases (ILD), especially Idiopathic Pulmonary Fibrosis (IPF) and Progressive Pulmonary Fibrosis (PPF), remains a challenge. …”
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FBR2 modulates ferroptosis via the SIRT3/p53 pathway to ameliorate pulmonary fibrosis by Yu Cheng, Yang Jiao, Wan Wei, Mengjia Kou, Yaodong Cai, Yang Li, Hao Li, Tonghua Liu

“…BackgroundIdiopathic Pulmonary Fibrosis (IPF), an interstitial lung disease of unknown etiology, remains incurable with current therapies, which fail to halt disease progression or restore lung function. …”
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Role of IPF genetic risk loci in post-COVID-19 lung abnormalities: a cohort study by James Johnston, Christopher Carlsten, Christopher J Ryerson, Alyson W Wong, Cameron J Hague, Julia Yang, Janice Leung, Daniel-Costin Marinescu, Aditi Shah, Darra Murphy

“…Similar parenchymal changes are observed in idiopathic pulmonary fibrosis (IPF). We investigated whether common genetic risk factors in IPF are associated with developing lung parenchymal abnormalities following severe COVID-19 disease.Methods Consecutive adults hospitalised for laboratory-confirmed COVID-19 infection were prospectively recruited from March to May 2020. …”
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Novel functional eQTL-SNPs associated with susceptibility to occupational pulmonary fibrosis: A multi-stage study by Rui Zhao, Xiaobo Tao, Wendi Zhang, Siqi Li, Shenxuan Zhou, Anhui Ning, Zhenyu Li, Minjie Chu, Wei Wang, Junhong Jiang

“…Aim: Identifying the common functional single-nucleotide polymorphisms (SNPs) that can both affect the susceptibility to idiopathic pulmonary fibrosis (IPF) and silicosis. Methods: We first integrated the genome-wide association studies (GWASs) of IPF and silicosis to obtain the shared SNPs. …”
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TWEAK-Fn14 signaling protects mice from pulmonary fibrosis by inhibiting fibroblast activation and recruiting pro-regenerative macrophages by Li Liu, Pei Wu, Yuqi Wei, Meng Lu, Haiyan Ge, Ping Wang, Jianlong Sun, Tiffany Horng, Xiucheng Liu, Xiaoyong Shen, Lingyun Sun, Ying Xi

“…Summary: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease characterized by excess accumulation of the extracellular matrix (ECM). …”
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3D-CT-derived lung volumes and mortality risk in patients with fibrotic hypersensitivity pneumonitis by Shusuke Yazawa, Yuzo Suzuki, Yuko Tanaka, Koshi Yokomura, Masato Kono, Dai Hashimoto, Atsuki Fukada, Yusuke Inoue, Hideki Yasui, Hironao Hozumi, Masato Karayama, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Naoki Inui, Takafumi Suda

“…The 3D-CT LV was compared with those of 42 controls and 140 patients with idiopathic pulmonary fibrosis (IPF). Results: Compared to patients with fibrotic-HP, the standardized total LV was significantly higher in controls and patients with non-fibrotic-HP and was similar in patients with IPF. …”
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JUNB O‐GlcNAcylation‐Mediated Promoter Accessibility of Metabolic Genes Modulates Distinct Epithelial Lineage in Pulmonary Fibrosis by Marie‐Therese Bammert, Meshal Ansari, Leoni Haag, Zuhdi Ahmad, Victoria Schröder, Joseph Birch, Diana Santacruz, Werner Rust, Coralie Viollet, Benjamin Strobel, Alec Dick, Florian Gantner, Holger Schlüter, Fidel Ramirez, Muriel Lizé, Matthew J. Thomas, Huy Q. Le

“…Abstract Idiopathic pulmonary fibrosis (IPF) is a lethal disease with substantial unmet medical needs. …”
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The bronchoalveolar lavage fluid CD44 as a marker for pulmonary fibrosis in diffuse parenchymal lung diseases by Magda Suchankova, Magda Suchankova, Eszter Zsemlye, Eszter Zsemlye, Jan Urban, Peter Baráth, Lenka Kohútová, Barbara Siváková, Barbara Siváková, Martina Ganovska, Elena Tibenska, Kinga Szaboova, Eva Tedlova, Dominik Juskanic, Dominik Juskanic, Kristina Kluckova, Kristina Kluckova, Michaela Kardohelyova, Tetiana Moskalets, Anna Ohradanova-Repic, Patrik Babulic, Maria Bucova, Vladimir Leksa

“…Among many pathological phenotypes, pulmonary fibrosis is the most devastating and represents a characteristic sign of idiopathic pulmonary fibrosis (IPF). Despite a poor prognosis brought by pulmonary fibrosis, there are no specific diagnostic biomarkers for the initial development of this fatal condition. …”
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Proteomic profiling of bronchoalveolar lavage fluid uncovers protein clusters linked to survival in idiopathic forms of interstitial lung disease by Linh T. Ngo, Michaella J. Rekowski, Devin C. Koestler, Takafumi Yorozuya, Atsushi Saito, Imaan Azeem, Alexis Harrison, M. Kristen Demoruelle, Jonathan Boomer, Bryant R. England, Paul Wolters, Philip L. Molyneaux, Mario Castro, Joyce S. Lee, Joshua J. Solomon, Koji Koronuma, Michael P. Washburn, Scott M. Matson

“…Background Idiopathic interstitial pneumonias (IIPs), such as idiopathic pulmonary fibrosis and interstitial pneumonia with autoimmune features, present diagnostic and therapeutic challenges due to their heterogeneous nature. …”
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Endocytic recycling is central to circadian collagen fibrillogenesis and disrupted in fibrosis by Joan Chang, Adam Pickard, Jeremy A Herrera, Sarah O'Keefe, Richa Garva, Matthew Hartshorn, Anna Hoyle, Lewis Dingle, John Knox, Thomas A Jowitt, Madeleine Coy, Jason Wong, Adam Reid, Yinhui Lu, Cédric Zeltz, Rajamiyer V Venkateswaran, Patrick T Caswell, Stephen High, Donald Gullberg, Karl E Kadler

“…Endocytic recycling of collagen-I was enhanced in human fibroblasts isolated from idiopathic pulmonary fibrosis, where VPS33B and integrin α11 subunit were overexpressed at the fibrogenic front; this correlation between VPS33B, integrin α11 subunit, and abnormal collagen deposition was also observed in samples from patients with chronic skin wounds. …”
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Evaluating the Diagnostic Value of Lymphocyte Subsets in Bronchoalveolar Lavage Fluid and Peripheral Blood Across Various Diffuse Interstitial Lung Disease Subtypes by Sonoko Harada, Motoyasu Kato, Kazuyuki Nakagome, Hitoshi Sasano, Yuki Tanabe, Tomohito Takeshige, Yuuki Sandhu, Kei Matsuno, Shoko Ueda, Sumiko Abe, Takayasu Nishimaki, Shun Shinomiya, Jun Ito, Sachiko Miyake, Ko Okumura, Makoto Nagata, Kazuhisa Takahashi, Norihiro Harada

“…This study examines lymphocyte subsets in BAL fluid and peripheral blood of 56 patients with diffuse ILD, excluding idiopathic pulmonary fibrosis (IPF), who underwent BAL for diagnostic purposes. …”
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The fibronectin-targeting PEG-FUD imaging probe shows enhanced uptake during fibrogenesis in experimental lung fibrosis by Thomas J. Harr, Nikesh Gupta, Babita Rahar, Kristen Stott, Yadira Medina-Guevara, Metti K. Gari, Angie T. Oler, Ivy Sohee McDermott, Hye Jin Lee, Morteza Rasoulianboroujeni, Ashley M. Weichmann, Amir Forati, Kelsey Holbert, Trevor S. Langel, Kade W. Coulter, Brian M. Burkel, Bianca R. Tomasini-Johansson, Suzanne M. Ponik, Jonathan W. Engle, Reinier Hernandez, Glen S. Kwon, Nathan Sandbo, Ksenija Bernau

“…Abstract Progressive forms of interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), are deadly disorders lacking non-invasive biomarkers for assessment of early disease activity, which presents a major obstacle in disease management. …”
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Acute exacerbations in patients with progressive pulmonary fibrosis by Michael Kreuter, Elizabeth A. Belloli, Elisabeth Bendstrup, Stefania Cerri, Kevin R. Flaherty, Shane Shapera, Jin Woo Song, Heiko Mueller, Klaus B. Rohr, Yasuhiro Kondoh, on behalf of the INBUILD trial investigators, S. Quadrelli, M. Otaola, M.A. Bergna, P. Elias, G. Arce, A. Cazaux, J. Guiot, B. Bondue, C. Dahlqvist, L. Homik, S. Shapera, A. Cantin, M. Kolb, M. Salinas Fénero, R. Maturana Rozas, A. Silva Orellana, Z. Xu, Q. Luo, J. Kang, H. Cai, S. Marchand-Adam, E. Bergot, A. Gamez-Dubuis, F. Riviere, R. Kessler, H. Nunes, C. Marquette, L. Wemeau, S. Jouneau, F. Lebargy, B. Crestani, V. Cottin, M. Reynaud-Gaubert, S. Blaas, F. Bonella, W. Randerath, J. Hetzel, D. Koschel, M. Kreuter, A. Prasse, D. Skowasch, S. Stieglitz, R. Refini, S. Cerri, A. Pesci, S. Tomassetti, C. Vancheri, F. Varone, N. Sakamoto, S. Abe, H. Hayashi, T. Saito, T. Suda, H. Kitamura, M. Okamoto, Y. Kondoh, S. Makino, T. Takeuchi, Y. Yamada, C. Kono, Y. Inoue, H. Sugiura, K. Kishi, H. Takaya, H. Yamauchi, K. Ichikado, K. Tomii, H. Takahashi, S. Izumi, T. Kawamura, Y. Nishioka, Y. Miyazaki, J.W. Song, J.S. Park, Y. Kim, E. Jassem, J. Kus, W. Piotrowski, A. Barczyk, D. Ziora, E. Bazdyrev, S. Moiseev, S. Avdeev, M. Ilkovich, V. Yakusevich, C. Valenzuela, O. Acosta, M. Martínez, L. Gómez, M. Molina-Molina, D.M. Castillo Villegas, M. Aburto, J.A. Rodríguez Portal, A. Villar, A. León Jiménez, J. Sauleda, M. Arias, P. Beirne, H. Stone, B. Hope-Gill, N. Hirani, N. Chaudhuri, A. Gifford, L. Jones, L. Morrison, D. Antin-Ozerkis, N. Bhatt, T. Kulkarni, T. Moua, N. Ettinger, L. Pitts, S. Veeraraghavan, M. Padilla, E.R. Fernández Pérez, G. Giessel, M. Strek, S. Danoff, J. Burk, M. Rossman, N. Patel, E. Belloli, D. Hotchkin, S. Weigt, M.B. Scholand, R. Kaner, B. Sigal, Z. Safdar, L. Tolle, R. Martinez, M. Glassberg, R. Hallowell, J. Golden, M. Schwartz, E. Britt, L. Morrow, Y. Mageto, K. Buch, S. Chaaban, H. Poonyagariyagorn, D. Dilling, O. Shlobin, K. Thavarajah, A. Nambiar, I. Rosas, R. Bascom, J. Oldham, S. Schmidt, J. Dematte D'Amico, J. Falk, C. Glazer, G. Criner

“…Methods Patients with progressive fibrosing ILDs other than idiopathic pulmonary fibrosis (IPF) were randomised to receive nintedanib or placebo. …”
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TRAIL-Dependent Resolution of Pulmonary Fibrosis by David M. Habiel, Ana Paula Moreira, Ugur B. Ismailoglu, Michael P. Dunleavy, Karen A. Cavassani, Nico van Rooijen, Ana Lucia Coelho, Cory M. Hogaboam

“…Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease characterized by the persistence of activated myofibroblasts resulting in excessive deposition of extracellular matrix proteins and profound tissue remodeling. …”
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