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Respirology 2022 Jun 28. Epub 2022 Jun 28.

Faculty of Medicine, The University of Queensland, Brisbane, Queensland, Australia.

Curr Opin Gastroenterol 2022 Jul;38(4):411-416

Advanced Lung Disease Specialists, Baylor University Medical Center, Dallas, Texas, USA.

Purpose Of Review: Idiopathic pulmonary fibrosis (IPF) is a prevalent subset of interstitial lung disease (ILD) that often progresses to require lung transplantation. Gastroesophageal reflux disease (GERD) is common in the IPF population, and GER-related micro-aspiration appears to be an important risk factor for IPF pathogenesis and for the deterioration of transplanted lung function.

Recent Findings: Many patients with IPF have elevated esophageal acid exposure on reflux testing despite having no or minimal symptoms. Read More

Lancet Respir Med 2022 Jun 24. Epub 2022 Jun 24.

Medical School, National Kapodistrian University of Athens and Athens Medical Center, Athens 15125, Greece. Electronic address:

Respir Med 2022 Jun 21;200:106922. Epub 2022 Jun 21.

Center of Medicine and Clinical Research, Division of Respiratory Medicine, Kuopio University Hospital, POB 100, 70029, Kuopio, Finland.

Background: The use of a transbronchial lung cryobiopsy (TBLC) is increasing as a diagnostic method of interstitial lung diseases (ILD). This study aimed to evaluate risk factors associated with clinically significant complications of TBLC in ILD patients.

Methods: Patients referred to Kuopio or Tampere university hospitals, in Finland, for a suspected ILD were included. Read More

Clin Transl Immunology 2022 16;11(6):e1398. Epub 2022 Jun 16.

Pediatric Pneumology, Allergology and Neonatology Hannover Medical School Hannover Germany.

Objectives: The contribution of adaptive innate lymphocytes to IL-17A and IL-22 secretion at the end stage of chronic lung diseases remains largely unexplored. In order to uncover tissue- and disease-specific secretion patterns, we compared production patterns of IL-17A and IL-22 in three different human end-stage lung disease entities.

Methods: Production of IL-17A, IL-22 and associated cytokines was assessed in supernatants of re-stimulated lymphocytes by multiplex assays and multicolour flow cytometry of conventional T cells, iNKT cells, γδ T cells and innate lymphoid cells in bronchial lymph node and lung tissue from patients with emphysema ( = 19), idiopathic pulmonary fibrosis ( = 14) and cystic fibrosis ( = 23), as well as lung donors ( = 17). Read More

Comput Intell Neurosci 2022 16;2022:5607358. Epub 2022 Jun 16.

The First People's Hospital of Chongqing Liangjiang New Area, Chongqing 401121, China.

Objective: Using data investigation, the microbiology of bacterial infection in patients with pulmonary infection was discussed, and its clinical characteristics were analyzed.

Methods: The clinical data of 160 patients with pulmonary infection in our hospital from March 2019 to March 2021 were collected and analyzed. Blood samples were collected and cultured, and the pathogens were identified. Read More

Front Med (Lausanne) 2022 9;9:861076. Epub 2022 Jun 9.

Department of Pulmonary Medicine, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.

A timely, confirmed diagnosis of Idiopathic Pulmonary Fibrosis (IPF) has a significant impact on the evolution of the disease. The current model of care in the Lazio region (in Italy) was assessed on the basis of real-world data provided by the four reference centers responsible for diagnosing and treating IPF. The 5-year, population-based, retrospective longitudinal study provided the data that is at the basis of the current proposal for a new clinical and therapeutic pathway (DTCP) and has been shared with regional decision makers. Read More

Front Genet 2022 9;13:890530. Epub 2022 Jun 9.

The First Clinical Medical College, The First Affiliated Hospital of Guangxi Medical University, Nanning, China.

Idiopathic pulmonary fibrosis (IPF) is the interstitial lung disease with the highest incidence and mortality. The lack of specific markers results in limited treatment methods for IPF patients. Numerous prognostic signatures represented effective indexes in predicting the survival of patients in various diseases; however, little is investigated on their application in IPF. Read More

Rev Mal Respir 2022 Jun 22. Epub 2022 Jun 22.

Service d'anatomie-pathologique, groupement hospitalier est, HCL, Bron, France.

Background: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated.

Methods: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. Read More

Curr Opin Pulm Med 2022 Jul;28(4):296-302

Respiratory Disease Unit, Department of Cardiac Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy.

Purpose Of Review: Growing evidence suggests that ageing-associated alterations occur in both idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD). Here, we review the most recent literature on dysregulated ageing pathways in IPF and COPD and discuss how they may contribute to disease pathogenesis.

Recent Findings: Recent studies have shown that alveolar epithelial type II (ATII) cells undergo premature senescence under stress and that senescent ATII cells promote lung fibrogenesis. Read More

PLoS One 2022 24;17(6):e0270297. Epub 2022 Jun 24.

Department of Gastroenterology, Dongfang Hospital, Beijing University of Chinese Medicine, Beijing, China.

Introduction: Inflammatory bowel disease is a relapsing chronic gastrointestinal inflammatory disease. Idiopathic pulmonary fibrosis is a rare but serious extraintestinal pulmonary manifestation of inflammatory bowel disease. However, the relationship between these two conditions is unclear. Read More

Front Pharmacol 2022 7;13:771031. Epub 2022 Jun 7.

Xingzhi College, Zhejiang Normal University, Lanxi, China.

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal interstitial lung disease characterized by myofibroblast accumulation and extracellular matrix deposition, which lead to irreversible damage of the lung's architecture and the formation of fibrotic lesions. IPF is also a sequela in serious patients with the coronavirus disease 2019 (COVID-19). The molecular mechanisms under pulmonary fibrosis remain unclear, and there is no satisfactory treatment currently available. Read More

ERJ Open Res 2022 Apr 20;8(2). Epub 2022 Jun 20.

Dept for BioMedical Research DBMR, Dept of Pulmonary Medicine, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.

Patients with progressive fibrosing interstitial lung diseases (fILD) have increased morbidity and mortality. Lung fibrosis can be associated with lung cancer. The pathogenesis of both diseases shows similarities, although not all mechanisms are understood. Read More

Cureus 2022 Jun 18;14(6):e26072. Epub 2022 Jun 18.

Department of Pediatrics, Al Qassimi Women's and Children's Hospital, Sharjah, ARE.

Interstitial lung disease (ILD) is a rare disease defined as a specific type of chronic fibrosing interstitial pneumonitis whose effects are limited to the lung. Nonspecific interstitial pneumonia (NSIP) was defined as a histopathological form that can be seen in the presence of large different clinical and radiological features. The exact role of thyroid hormone in the pathogenetic mechanism of idiopathic interstitial pneumonitis (IIP) is unclear. Read More

Life (Basel) 2022 Jun 14;12(6). Epub 2022 Jun 14.

Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), 08036 Barcelona, Spain.

Coronavirus disease 2019 (COVID-19) is a pandemic respiratory disease associated with high morbidity and mortality. Although many patients recover, long-term sequelae after infection have become increasingly recognized and concerning. Among other sequelae, the available data indicate that many patients who recover from COVID-19 could develop fibrotic abnormalities over time. Read More

Int J Mol Sci 2022 Jun 14;23(12). Epub 2022 Jun 14.

Laboratorio de Biología Molecular, Instituto Nacional de Enfermedades Respiratorias (INER) "Ismael Cosío Villegas", Calz. Tlalpan 4502, Col. Sección XVI, Mexico City 14080, Mexico.

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disease with high mortality and unclear etiology. Previous evidence supports that the origin of this disease is associated with epigenetic alterations, age, and environmental factors. IPF initiates with chronic epithelial lung injuries, followed by basal membrane destruction, which promotes the activation of myofibroblasts and excessive synthesis of extracellular matrix (ECM) proteins, as well as epithelial-mesenchymal transition (EMT). Read More

Cells 2022 06 20;11(12). Epub 2022 Jun 20.

Department of Medicine II, Internal Medicine, Pulmonary and Critical Care, Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), Justus-Liebig University Giessen, 35392 Giessen, Germany.

Insulin-like growth factor (IGF) signaling controls the development and growth of many organs, including the lung. Loss of function of or its receptor impairs lung development and leads to neonatal respiratory distress in mice. Although many components of the IGF signaling pathway have shown to be dysregulated in idiopathic pulmonary fibrosis (IPF), the expression pattern of such components in different cellular compartments of the developing and/or fibrotic lung has been elusive. Read More

Biomolecules 2022 Jun 13;12(6). Epub 2022 Jun 13.

Department of Biology, University of Illinois at Springfield, Springfield, IL 62703, USA.

Reactive oxygen species (ROS) can be beneficial or harmful in health and disease. While low levels of ROS serve as signaling molecules to regulate vascular tone and the growth and proliferation of endothelial cells, elevated levels of ROS contribute to numerous pathologies, such as endothelial dysfunctions, colon cancer, and fibrosis. ROS and their cellular sources have been extensively studied as potential targets for clinical intervention. Read More

Biomedicines 2022 Jun 15;10(6). Epub 2022 Jun 15.

Department of Pharmacology, Faculty of Medicine, University of Valencia, 46010 Valencia, Spain.

In the lungs, fibrosis is a growing clinical problem that results in shortness of breath and can end up in respiratory failure. Even though the main fibrotic disease affecting the lung is idiopathic pulmonary fibrosis (IPF), which affects the interstitial space, there are many fibrotic events that have high and dangerous consequences for the lungs. Asthma, chronic obstructive pulmonary disease (COPD), excessive allergies, clearance of infection or COVID-19, all are frequent diseases that show lung fibrosis. Read More

Chest 2022 Jun 20. Epub 2022 Jun 20.

Department of Medicine, University of British Columbia, Vancouver, BC, Canada; Centre for Heart Lung Innovation, St. Paul's Hospital, Vancouver, BC, Canada.

Recent clinical practice guidelines have addressed the diagnosis of idiopathic pulmonary fibrosis (IPF) and fibrotic hypersensitivity pneumonitis (fHP). These disease-specific guidelines were developed independently, without clear direction on how to concurrently apply their respective recommendations within a single patient, where discrimination between these two fibrotic interstitial lung diseases represents a frequent diagnostic challenge. The objective of this document, created by an international group of experts, was to suggest a pragmatic approach on how to apply existing guidelines to distinguish IPF and fHP. Read More

Radiologie (Heidelb) 2022 Jun 23. Epub 2022 Jun 23.

Universitätsklinik für Radiologie und Nuklearmedizin, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich.

Clinical Issue: Smoking-related interstitial lung diseases are a heterogeneous group of pulmonary abnormalities. The correct diagnosis has prognostic and therapeutic implications. This article introduces the most common smoking-related interstitial lung diseases and describes a structured approach to support the diagnostic workflow. Read More

Biol Methods Protoc 2022 30;7(1):bpac013. Epub 2022 May 30.

Faculty of Health and Behavioural Sciences, School of Health and Rehabilitation Sciences, The University of Queensland, St Lucia, QLD 4072, Australia.

SARS-CoV-2, the virus that causes COVID-19, is a current concern for people worldwide. The virus has recently spread worldwide and is out of control in several countries, putting the outbreak into a terrifying phase. Machine learning with transcriptome analysis has advanced in recent years. Read More

Sci Adv 2022 06 22;8(25):eabn7162. Epub 2022 Jun 22.

Engineering Research Center of Cell and Therapeutic Antibody, Ministry of Education, and School of Pharmacy, Shanghai Jiao Tong University, 800 Dongchuan Road, Shanghai 200240, PR China.

Interleukin-11 (IL-11) is a profibrotic cytokine essential for the differentiation of fibroblasts into collagen-secreting, actin alpha 2, smooth muscle-positive (ACTA2) myofibroblasts, driving processes underlying the pathogenesis of idiopathic pulmonary fibrosis (IPF). Here, we developed an inhalable and mucus-penetrative nanoparticle (NP) system incorporating siRNA against ([email protected] NPs) and investigated therapeutic potential for the treatment of IPF. NPs are formulated through self-assembly of a biodegradable PLGA-PEG diblock copolymer and a self-created cationic lipid-like molecule G0-C14 to enable efficient transmucosal delivery of si. Read More

Expert Rev Respir Med 2022 Jun 29:1-9. Epub 2022 Jun 29.

Department of Pulmonary and Critical Care Medicine, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai, P.R.China.

Background: Idiopathic pulmonary fibrosis (IPF) can combine with emphysema, a condition termed as IPF with emphysema (IPFE). We compared the clinical, radiologic, and physiologic features of IPF and IPFE.

Research Design And Methods: Newly diagnosed IPF    and IPFE    patients were recruited between January 2018 and September 2020. Read More

Korean J Intern Med 2022 Jun 22. Epub 2022 Jun 22.

Division of Allergy and Respiratory Medicine, Department of Internal Medicine, Soonchunhyang University Bucheon Hospital, Bucheon, Korea.

Background/aims: Neutrophilia is frequently observed in bronchoalveolar lavage fluid (BALF) of idiopathic pulmonary fibrosis (IPF) patients. Granulocyte colony-stimulating factor (G-CSF) is a potent neutrophil-activating glycoprotein. However, the clinical implications of G-CSF remain poorly understood. Read More

Am J Respir Crit Care Med 2022 Jun 21. Epub 2022 Jun 21.

Vanderbilt University Medical Center, 12328, Allergy, Pulmonary and Critical Care Medicine, Nashville, Tennessee, United States;

Rationale: Although persistent fibroblast activation is a hallmark of idiopathic pulmonary fibrosis (IPF), mechanisms regulating persistent fibroblast activation in the lungs have not been fully elucidated.

Objective: Based on our observation that lung fibroblasts express thromboxane-prostanoid receptor (TBXA2R) during fibrosis, we investigated the role of TBXA2R signaling in fibrotic remodeling.

Methods: We identified TBXA2R expression in lungs of IPF patients and mice, and studied primary mouse and human lung fibroblasts to determine the impact of TBXA2R signaling on fibroblast activation. Read More

Nurs Open 2022 Jun 20. Epub 2022 Jun 20.

Graduate School of Nursing Science, St. Luke's International University, Tokyo, Japan.

Aims: This study evaluated the acceptability of a dignity-centred palliative care programme for people with idiopathic pulmonary fibrosis by converging perceptions of living with idiopathic pulmonary fibrosis qualitative data and quantitative data.

Design: The qualitative-driven mixed methods research addressed the study aim by using a convergent design. This single arm, non-randomized study used purposive sampling. Read More

Respir Res 2022 Jun 20;23(1):162. Epub 2022 Jun 20.

Department of Pulmonary Medicine, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, The Netherlands.

Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and severe disease characterized by excessive matrix deposition in the lungs. Macrophages play crucial roles in maintaining lung homeostasis but are also central in the pathogenesis of lung diseases like pulmonary fibrosis. Especially, macrophage polarization/activation seems to play a crucial role in pathology and epigenetic reprograming is well-known to regulate macrophage polarization. Read More

Front Pharmacol 2022 2;13:899469. Epub 2022 Jun 2.

Respiratory Medicine, Allergology, and Palliative Medicine, Department of Clinical Sciences Lund, Lund University and Skåne University Hospital, Lund, Sweden.

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease causing irreparable scarring of lung tissue, with most patients succumbing rapidly after diagnosis. The mevalonate pathway, which is involved in the regulation of cell proliferation, survival, and motility, is targeted by the bisphosphonate zoledronic acid (ZA). The aim of this study was to assess the antifibrotic effects of ZA and to elucidate the mechanisms by which potential IPF treatment occurs. Read More

Front Pharmacol 2022 3;13:918771. Epub 2022 Jun 3.

Department of Rehabilitation Medicine, West China Hospital, Sichuan University, Chengdu, China.

Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease of unknown origin that usually results in death from secondary respiratory failure within 2-5 years of diagnosis. Recent studies have identified key roles of cytokine and growth factor pathways in the pathogenesis of IPF. Although there have been numerous clinical trials of drugs investigating their efficacy in the treatment of IPF, only Pirfenidone and Nintedanib have been approved by the FDA. Read More