Article
Critical Care Medicine
Yosafe Wakwaya, Deepa Ramdurai, Jeffrey J. Swigris
Summary: Chronic cough in patients with IPF may involve multiple mechanisms, including comorbid conditions and IPF itself. Diagnostic and management approaches need to be multi-faceted, but may lack robust data support.
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.
Review
Medicine, General & Internal
Alba Mulet, Jaime Signes-Costa
Summary: Idiopathic pulmonary fibrosis is a lung disease with unknown causes, which has a poor prognosis. Telomeric shortening may play an important role in its pathogenesis, but mutations in telomere-related genes are not always present. Telomere shortening can serve as a biomarker for disease prognosis independently of other clinical factors.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Pharmacology & Pharmacy
Xudan Yang, Zhihao Xu, Songhua Hu, Juan Shen
Summary: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive interstitial lung disease without a known cause. Current approved drugs for IPF, such as Pirfenidone and Nintedanib, can slow down the decline in lung function and reduce the risk of acute worsening. However, they cannot alleviate symptoms or improve overall survival. The development of new, safe, and effective drugs is necessary. Previous studies have shown that cyclic nucleotides and phosphodiesterase inhibitors may be potential targets for treating pulmonary fibrosis. This paper reviews the progress in research on PDE inhibitors and provides insights for the development of anti-pulmonary fibrosis drugs.
FRONTIERS IN PHARMACOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Claudia De Vitis, Michela D'Ascanio, Andrea Sacconi, Dario Pizzirusso, Valentina Salvati, Massimiliano Mancini, Giorgia Scafetta, Roberto Cirombella, Francesca Ascenzi, Sara Bruschini, Antonella Esposito, Silvia Castelli, Claudia Salvucci, Leonardo Teodonio, Bruno Sposato, Angela Catizone, Arianna Di Napoli, Andrea Vecchione, Gennaro Ciliberto, Salvatore Sciacchitano, Alberto Ricci, Rita Mancini
Summary: The study revealed that the B4GALT1 gene is overexpressed in IPF patients and human cell cultures, indicating it could serve as a potential novel marker for this disease.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Pharmacology & Pharmacy
Chengsheng Yin, Huikang Xie, Xian He, Yuan Zhang, Aihong Zhang, Huiping Li
Summary: This study aimed to investigate the occurrence of small airway dysfunction (SAD) comorbidity in idiopathic pulmonary fibrosis (IPF) and its impact on survival. The study found that 34.57% of IPF patients were diagnosed with SAD, and comorbid SAD was an independent risk factor for mortality in IPF patients.
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Medicine, General & Internal
Theodoros Karampitsakos, Brenda M. Juan-Guardela, Argyris Tzouvelekis, Jose D. Herazo-Maya
Summary: Idiopathic pulmonary fibrosis (IPF) is a highly heterogeneous and lethal chronic lung disease. Precision medicine approaches have not been effectively implemented in IPF clinical practice, despite the existence of numerous diagnostic, prognostic, and theragnostic biomarker candidates.
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
Biochemistry & Molecular Biology
Hamid Mattoo, Shiv Pillai
Summary: This review focuses on the pathogenesis of fibrotic diseases, mainly looking at triggers, progression processes, therapies, and therapeutic trials. It primarily discusses idiopathic pulmonary fibrosis and systemic sclerosis, highlighting the contributions of inflammatory mechanisms and adaptive immunity respectively.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Review
Pharmacology & Pharmacy
Qianru Mei, Zhe Liu, He Zuo, Zhenhua Yang, Jing Qu
Summary: Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease with limited understanding of its pathogenesis. Recent studies suggest that sustained lung epithelial injury and fibroblast differentiation play key roles in the development of IPF. Current treatments can slow disease progression but cannot cure it.
FRONTIERS IN PHARMACOLOGY
(2022)
Review
Pharmacology & Pharmacy
Mingyao Sun, Yangyang Sun, Ziru Feng, Xinliang Kang, Weijie Yang, Yongan Wang, Yuan Luo
Summary: IPF is a progressive disease characterized by an inexorable decline in lung function, involving multiple positive feedback loops and the crucial role of the Hippo/YAP signaling pathway. YAP/TAZ plays a key role in pulmonary fibrosis and alveolar regeneration by regulating cell proliferation and extracellular matrix deposition through different signaling pathways.
PHARMACOLOGICAL RESEARCH
(2021)
Review
Biochemistry & Molecular Biology
Paschalis Ntolios, Vassilios Tzilas, Evangelos Bouros, Eleni Avdoula, Ioannis Karakasiliotis, Demosthenes Bouros, Paschalis Steiropoulos
Summary: The interest in the lung microbiome and virome and their contribution to the pathogenesis of idiopathic pulmonary fibrosis (IPF) has been increasing. Higher microbial burden is associated with worse prognosis but no specific microbe has been identified to contribute to this. Infection is considered a cause of acute exacerbation of IPF, but the association of viral infection with AE-IPF has not been established.
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
Respiratory System
Andrew Shapanis, Mark G. Jones, James Schofield, Paul Skipp
Summary: A machine learning model and multiple datasets were used to accurately predict idiopathic pulmonary fibrosis (IPF) and identified distinct subphenotypes of the disease.
Review
Medicine, General & Internal
Francisco Leon-Roman, Claudia Valenzuela, Maria Molina-Molina
Summary: Idiopathic pulmonary fibrosis is a chronic progressive fibrosing interstitial pneumonia of unknown cause. The disease can be diagnosed through radiological and histological findings and there are currently antifibrotic drugs available for slowing its progression. A multidisciplinary approach can improve patients' overall condition.
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
Respiratory System
Irina R. Strambu, Christian A. Seemayer, Liesbeth M-C. A. Fagard, Paul A. Ford, Tom A. K. Van der Aa, Angela A. de Haas-Amatsaleh, Vikas Modgill, Eva Santermans, Eric N. Sondag, Eric G. Helmer, Toby M. Maher, Ulrich Costabel, Vincent Cottin
Summary: This study assessed the efficacy, safety, and tolerability of GLPG1205 for the treatment of idiopathic pulmonary fibrosis (IPF). The results showed that GLPG1205 did not have a significant difference in the decline of lung function compared to placebo, but it had a poorer safety and tolerability profile.
EUROPEAN RESPIRATORY JOURNAL
(2023)
Article
Critical Care Medicine
Iain Stewart, Joseph Jacob, Peter M. George, Philip L. Molyneaux, Joanna C. Porter, Richard J. Allen, Shahab Aslani, J. Kenneth Baillie, Shaney L. Barratt, Paul Beirne, Stephen M. Bianchi, John F. Blaikley, James D. Chalmers, Rachel C. Chambers, Nazia Chadhuri, Christopher Coleman, Guilhem Collier, Emma K. Denneny, Annemarie Docherty, Omer Elneima, Rachael A. Evans, Laura Fabbri, Michael A. Gibbons, Fergus Gleeson, Bibek Gooptu, Neil J. Greening, Beatriz Guillen Guio, Ian P. Hall, Neil A. Hanley, Victoria Harris, Ewen M. Harrison, Melissa Heightman, Toby E. Hillman, Alex Horsley, Linzy Houchen-Wolloff, Ian Jarrold, Simon R. Johnson, Mark G. Jones, Fasihul Khan, Rod Lawson, Olivia Leavy, Nazir Lone, Michael Marks, Hamish McAuley, Puja Mehta, Dhruv Parekh, Karen Piper Hanley, Manuela Plate, John Pearl, Krisnah Poinasamy, Jennifer K. Quint, Betty Raman, Matthew Richardson, Pilar Rivera-Ortega, Laura Saunders, Ruth Saunders, Malcolm G. Semple, Marco Sereno, Aarti Shikotra, A. John Simpson, Amisha Singapuri, David J. F. Smith, Mark Spears, Lisa G. Spencer, Stefan Stanel, David R. Thickett, A. A. Roger Thompson, Mathew Thorpe, Simon L. F. Walsh, Samantha Walker, Nicholas David Weatherley, Mark E. Weeks, Jim M. Wild, Dan G. Wootton, Chris E. Brightling, Ling-Pei Ho, Louise Wain, Gisli R. Jenkins
Summary: This study analyzed the data from the UK Interstitial Lung Disease Consortium (UKILD) post-COVID-19 study to estimate the prevalence of residual lung abnormalities in hospitalized COVID-19 patients. The results showed that approximately 11% of recovered COVID-19 patients had residual lung abnormalities, highlighting the need for long-term monitoring.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Article
Medicine, General & Internal
Felicity Liew, Shubha Talwar, Andy Cross, Brian J. Willett, Sam Scott, Nicola Logan, Matthew K. Siggins, Dawid Swieboda, Jasmin K. Sidhu, Claudia Efstathiou, Shona C. Moore, Chris Davis, Noura Mohamed, Jose Nunag, Clara King, A. A. Roger Thompson, Sarah L. Rowland-Jones, Annemarie B. Docherty, James D. Chalmers, Ling-Pei Ho, Alexander Horsley, Betty Raman, Krisnah Poinasamy, Michael Marks, Onn Min Kon, Luke Howard, Daniel G. Wootton, Susanna Dunachie, Jennifer K. Quint, Rachael A. Evans, Louise V. Wain, Sara Fontanella, Thushan I. de Silva, Antonia Ho, Ewen Harrison, J. Kenneth Baillie, Malcolm G. Semple, Christopher Brightling, Ryan S. Thwaites, Lance Turtle, Peter J. M. Openshaw
Summary: This study examined the nasal and plasma antibody responses in COVID-19 hospitalized patients one year after discharge and vaccination. The findings showed sustained elevated antibody responses in both nasal and plasma samples for at least 12 months, but the nasal antibody response was minimally influenced by vaccination. These findings highlight the importance of developing vaccines that enhance nasal immunity.
Article
Critical Care Medicine
Justin M. Oldham, Richard J. Allen, Jose M. Lorenzo-Salazar, Philip L. Molyneaux, Shwu-Fan Ma, Chitra Joseph, John S. Kim, Beatriz Guillen-Guio, Tamara Hernandez-Beeftink, Jonathan A. Kropski, Yong Huang, Cathryn T. Lee, Ayodeji Adegunsoye, Janelle Vu Pugashetti, Angela L. Linderholm, Vivian Vo, Mary E. Strek, Jonathan Jou, Adrian Munoz-Barrera, Luis A. Rubio-Rodriguez, Richard Hubbard, Nik Hirani, Moira K. B. Whyte, Simon Hart, Andrew G. Nicholson, Lisa Lancaster, Helen Parfrey, Doris Rassl, William Wallace, Eleanor Valenzi, Yingze Zhang, Josyf Mychaleckyj, Amy Stockwell, Naftali Kaminski, Paul J. Wolters, Maria Molina-Molina, Nicholas E. Banovich, William A. Fahy, Fernando J. Martinez, Ian P. Hall, Martin D. Tobin, Toby M. Maher, Timothy S. Blackwell, Brian L. Yaspan, R. Gisli Jenkins, Carlos Flores, Louise V. Wain, Imre Noth
Summary: This study conducted a staged genome-wide association study to identify and validate molecular determinants of survival in idiopathic pulmonary fibrosis (IPF). Four novel variants associated with IPF survival were identified, including one within the PCSK6 gene that reached genome-wide significance. Downstream analyses suggested that PCSK6 protein may play an important role in IPF progression.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
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)
Review
Respiratory System
Martin Kolb, Bruno Crestani, Toby M. Maher
Summary: Patients with interstitial lung disease can develop a progressive fibrosing phenotype with irreversible decline in lung function. Current therapies have limited efficacy and undesirable side-effects. PDE4B inhibitors show promise in stabilizing pulmonary function and improving tolerability. Further research is needed to evaluate their efficacy and safety in larger patient populations and longer treatment periods.
EUROPEAN RESPIRATORY REVIEW
(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
Medicine, General & Internal
Hamish J. C. McAuley, Rachael A. Evans, Charlotte E. Bolton, Christopher E. Brightling, James D. Chalmers, Annemarie B. Docherty, Omer Elneima, Paul L. Greenhaff, Ayushman Gupta, Victoria C. Harris, Ewen M. Harrison, Ling-Pei Ho, Alex Horsley, Linzy Houchen-Wolloff, Caroline J. Jolley, Olivia C. Leavy, Nazir I. Lone, William D. C. Man, Michael Marks, Dhruv Parekh, Krisnah Poinasamy, Jennifer K. Quint, Betty Raman, Matthew Richardson, Ruth M. Saunders, Marco Sereno, Aarti Shikotra, Amisha Singapuri, Sally J. Singh, Michael Steiner, Ai Lyn Tan, Louise Wain, Carly Welch, Julie Whitney, Miles D. Witham, Janet Lord, Neil J. Greening, HOSP-COVID Study Collaborat Grp
Summary: This study recruited COVID-19 survivors in the UK and objectively measured frailty using FFP. The results showed that frailty and pre-frailty are common after hospitalization with COVID-19, and comprehensive assessment and interventions targeting frailty are needed beyond the initial illness.
Article
Critical Care Medicine
Joseph L. Barnett, Toby M. Maher, Jennifer K. Quint, Alex Adamson, Zhe Wu, David J. F. Smith, Bhavin Rawal, Arjun Nair, Simon L. F. Walsh, Sujal R. Desai, Peter M. George, Maria Kokosi, Gisli Jenkins, Vasilis Kouranos, Elisabetta A. Renzoni, Alex Rice, Andrew G. Nicholson, Felix Chua, Athol U. Wells, Philip L. Molyneaux, Anand Devaraj
Summary: This study found that increased BAL lymphocyte proportion is rare in patients with extensive fibrosis or a UIP pattern on CT, and in patients without extensive fibrosis or a non-UIP pattern, an increased BAL lymphocyte proportion is associated with a lower likelihood of disease progression.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
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
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)
