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.
Review
Geriatrics & Gerontology
Gurparsad Singh Suri, Gurleen Kaur, Chandan Kumar Jha, Manish Tiwari
Summary: IPF is a chronic lung fibrosing disease with unclear pathogenesis and a poor prognosis. Recent advancements have shed light on the pathobiology of IPF, including specific pathologic and clinical features, and the molecular pathways involved. Internal and external factors play a role in disease disposition, with changes primarily occurring in epithelial cells and fibroblasts leading to profibrotic pathway activation. Treatment options include past and future medications and therapies.
EXPERIMENTAL GERONTOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Carlo Albera, Giulia Verri, Federico Sciarrone, Elena Sitia, Mauro Mangiapia, Paolo Solidoro
Summary: Interstitial lung diseases (ILDs) encompass a diverse group of rare and chronic respiratory disorders, with idiopathic pulmonary fibrosis (IPF) being the most studied member. Recent development of antifibrotic drugs has shown benefits in ILDs characterized by progressive fibrosis, leading to significant changes in management.
Review
Biochemistry & Molecular Biology
Francesco Amati, Anna Stainer, Veronica Polelli, Marco Mantero, Andrea Gramegna, Francesco Blasi, Stefano Aliberti
Summary: Pirfenidone and nintedanib are effective in slowing disease progression in interstitial lung diseases other than idiopathic pulmonary fibrosis. Two well-designed trials demonstrate the efficacy of nintedanib, while caution should be taken in interpreting the results of pirfenidone due to trial limitations. Ongoing randomized control trials aim to improve the quality of evidence in this field.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
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)
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)
Article
Biochemistry & Molecular Biology
Habib Layoun, Joelle Hajal, Youakim Saliba, Viviane Smayra, Bassem Habr, Nassim Fares
Summary: This study evaluated the effect of pirfenidone on idiopathic inflammatory myositis (IIM)-associated interstitial lung disease (ILD). The results showed that pirfenidone attenuated IIM-associated ILD by reducing inflammation and fibrosis. This study reveals the potential of pirfenidone to fine-tune TGF-beta 1 fibrotic signaling in IIM-associated ILD for the first time.
Article
Multidisciplinary Sciences
Yuzo Suzuki, Kazutaka Mori, Yuya Aono, Masato Kono, Hirotsugu Hasegawa, Koshi Yokomura, Hyogo Naoi, Hironao Hozumi, Masato Karayama, Kazuki Furuhashi, Noriyuki Enomoto, Tomoyuki Fujisawa, Yutaro Nakamura, Naoki Inui, Hidenori Nakamura, Takafumi Suda
Summary: The study evaluated the significance of the GAP index and BMI in predicting prognosis in patients with IPF receiving AFT.
SCIENTIFIC REPORTS
(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
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.
Article
Medicine, Research & Experimental
Vincent Cottin, Sonia Gueguen, Hilario Nunes, Stephane Jouneau, Bruno Crestani, Philippe Bonniaud, Lidwine Wemeau, Dominique Israel-Biet, Martine Reynaud-Gaubert, Anne Gondouin, Jacques Cadranel, Sylvain Marchand-Adam, Marie Chevereau, Isabelle Dufaure-Gare, Serge Amselem, Annick Clement
Summary: This study in France on IPF patients using pirfenidone capsules and tablets showed good acceptability of the tablet formulation among patients, suggesting possible advantages over the capsules. The study highlighted no new adverse events and lower discontinuation rates in the tablet and substitution subgroups compared to the capsules-only subgroup.
ADVANCES IN THERAPY
(2022)
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)
