Article
Immunology
Eleanor Valenzi, Tracy Tabib, Anna Papazoglou, John Sembrat, Humberto E. Trejo Bittar, Mauricio Rojas, Robert Lafyatis
Summary: IPF and SSc-ILD exhibit differences in cell types and pathways, providing new insights into the diseases.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Maria Martin-Lopez, Patricia E. Carreira
Summary: Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune dysregulation and progressive fibrosis. Interstitial lung disease (ILD) is the leading cause of death in patients with SSc. The management of SSc-ILD has shifted to include targeted biologic and antifibrotic therapies.
JOURNAL OF CLINICAL MEDICINE
(2023)
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
Biochemistry & Molecular Biology
Patricia Richter, Anca Cardoneanu, Nicoleta Dima, Ioana Bratoiu, Ciprian Rezus, Alexandra Maria Burlui, Damiana Costin, Luana Andreea Macovei, Elena Rezus
Summary: Interstitial lung disease (ILD) is a serious manifestation of connective tissue diseases (CTD) that requires thorough evaluation and treatment. The prevalence of ILD in systemic lupus erythematosus (SLE) is still debated, but it is crucial to exclude an overlap syndrome for an accurate diagnosis. Increasing the identification of SLE-associated ILD cases is important, and various treatment options are being proposed. Systemic sclerosis (SSc)-associated ILD is a leading cause of mortality and should be investigated in all SSc patients. Progress has been made in the treatment of ILD, with tyrosine kinases inhibitor Nintedanib showing promising results in reducing the progression rate compared to placebo.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Critical Care Medicine
Matthew Moll, Anna L. Peljto, John S. Kim, Hanfei Xu, Catherine L. Debban, Xianfeng Chen, Aravind Menon, Rachel K. Putman, Auyon J. Ghosh, Aabida Saferali, Mizuki Nishino, Hiroto Hatabu, Brian D. Hobbs, Julian Hecker, Gregory McDermott, Jeffrey A. Sparks, Louise V. Wain, Richard J. Allen, Martin D. Tobin, Benjamin A. Raby, Sung Chun, Edwin K. Silverman, Ana C. Zamora, Victor E. Ortega, Christine K. Garcia, R. Graham Barr, Eugene R. Bleecker, Deborah A. Meyers, Robert J. Kaner, Stephen S. Rich, Ani Manichaikul, Jerome I. Rotter, Josee Dupuis, George T. O'Connor, Tasha E. Fingerlin, Gary M. Hunninghake, David A. Schwartz, Michael H. Cho
Summary: This study found that a common genetic variant risk score plays an important role in identifying individuals at high risk of interstitial lung diseases and pulmonary fibrosis, complementing the MUC5B variant.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Joe E. Mouawad, Carol Feghali-Bostwick
Summary: Systemic sclerosis, also known as scleroderma, is an autoimmune disorder that affects the connective tissues and has a high mortality rate. Fibrosis, particularly in the lungs, is a hallmark of the disease and is currently the leading cause of death. Understanding the molecular mechanisms involved in lung fibrosis is essential for developing potential therapies to improve patient outcomes and quality of life.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
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
Yong Huang, Rob Guzy, Shwu-Fan Ma, Catherine A. Bonham, Jonathan Jou, Jefree J. Schulte, John S. Kim, Andrew J. Barros, Milena S. Espindola, Aliya N. Husain, Cory M. Hogaboam, Anne Sperling, Imre Noth
Summary: The contribution of central lung tissues to the pathogenesis of idiopathic pulmonary fibrosis (IPF) is still unknown. This study used RNA sequencing to investigate the relationship between cell types in IPF-central and IPF-peripheral lung tissues. The results showed that there were many shared upregulated genes between central and peripheral IPF, as well as some specifically upregulated genes in central IPF that were associated with the progression of fibrosis.
BMJ OPEN RESPIRATORY RESEARCH
(2023)
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
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
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)
Review
Biochemistry & Molecular Biology
Francesco Amati, Anna Stainer, Marco Mantero, Andrea Gramegna, Edoardo Simonetta, Giulia Suigo, Antonio Voza, Anoop M. Nambiar, Umberto Cariboni, Justin Oldham, Philip L. Molyneaux, Paolo Spagnolo, Francesco Blasi, Stefano Aliberti
Summary: Interstitial lung diseases are a complex group of diseases with unclear causes and mechanisms of progression. Recent research suggests that the lung microbiome may play a role in the development and progression of these diseases. However, the specific mechanisms are not yet understood. This review aims to discuss the role of altered lung microbiome in various interstitial lung diseases. Understanding the interaction between the host and microbiome in the lungs is a priority for research on these diseases.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(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)
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
Rheumatology
Kathleen Morrisroe, Dylan Hansen, Wendy Stevens, Laura Ross, Joanne Sahhar, Gene-Siew Ngian, Catherine L. Hill, Lauren Host, Jennifer Walker, Susanna Proudman, Mandana Nikpour
Summary: This study aimed to describe the frequency of progressive pulmonary fibrosis (PPF) in an incident cohort of systemic sclerosis (SSc)-related interstitial lung disease (ILD) and its impact on survival. The results showed that about one-third of the patients experienced PPF, and the presence of PPF was significantly associated with mortality. This suggests that some patients may benefit from earlier introduction of immunosuppressive and/or antifibrotic therapy.
