-
The New England Journal of Medicine Sep 2020
Review
Topics: Algorithms; Humans; Immunomodulation; Lung; Oxygen Inhalation Therapy; Prevalence; Pulmonary Fibrosis
PubMed: 32877584
DOI: 10.1056/NEJMra2005230 -
Chest Dec 2002Pulmonary fibrosis is a component of over 200 interstitial lung diseases. Some have known etiologies, however, for many diseases, the etiology remains unknown or... (Review)
Review
Pulmonary fibrosis is a component of over 200 interstitial lung diseases. Some have known etiologies, however, for many diseases, the etiology remains unknown or obscure. This brief review examines the prevalence and classification of these diseases, the approach to be taken for the investigation of a patient suspected of having pulmonary fibrosis, the indications for the performance of lung biopsy, and current thoughts concerning the pathogenesis of the idiopathic forms of fibrotic lung disease. A brief review of established and emerging therapeutic strategies is included.
Topics: Humans; Pulmonary Fibrosis
PubMed: 12475811
DOI: 10.1378/chest.122.6_suppl.334s -
Current Opinion in Pulmonary Medicine Sep 1996Idiopathic pulmonary fibrosis (IPF), or cryptogenic fibrosing alveolitis as it is known in the United Kingdom and Europe, is perhaps one of the most complex and...
Idiopathic pulmonary fibrosis (IPF), or cryptogenic fibrosing alveolitis as it is known in the United Kingdom and Europe, is perhaps one of the most complex and frustrating pulmonary disorders. The cause of this illness is unknown. Treatment often founders in a mire of discontent, dissatisfaction, and failure. In fact, the philosophic homily "If you do not know where you are going, then any road will take you there" in a curious way sums up the trials and tribulations of treating idiopathic interstitial fibrosis.
Topics: Adult; Female; Humans; Male; Middle Aged; Pulmonary Fibrosis
PubMed: 9363166
DOI: 10.1097/00063198-199609000-00001 -
Jornal Brasileiro de Pneumologia :... 2006Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These... (Review)
Review
Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These breakthroughs, combined with the increased general availability of therapeutic trials, hold the promise of a brighter future for idiopathic pulmonary fibrosis patients. For example, we now have a more comprehensive understanding of the diagnostic criteria and natural history of the disease. Several studies have shown that simple measurement of pulmonary physiology or gas exchange can be used to predict patient survival. By identifying several molecular pathways that play significant roles in the pathogenesis of idiopathic pulmonary fibrosis, investigators have produced a growing list of novel potential therapeutic targets for the disease. Several prospective, controlled therapeutic trials have been conducted. Others are ongoing or are still in the planning stages. These efforts have advanced our current knowledge of idiopathic pulmonary fibrosis and have raised new important questions, as well as having generated the interest and momentum needed to gain additional ground in the fight against this challenging disease. This article offers the reader a view of the recent advances in idiopathic pulmonary fibrosis research, with a focus on natural history, pathogenesis and treatment.
Topics: Clinical Trials as Topic; Humans; Prognosis; Pulmonary Fibrosis
PubMed: 17273615
DOI: No ID Found -
The American Journal of Nursing Apr 2003
Review
Topics: Disease Progression; Female; Home Care Services; Humans; Middle Aged; Oxygen Inhalation Therapy; Pulmonary Fibrosis; Risk Factors; United States
PubMed: 12677120
DOI: 10.1097/00000446-200304000-00016 -
The Journal of Clinical Investigation Aug 2004Pulmonary fibrosis is a devastating condition that leads to progressive lung destruction and scarring. Previous mechanistic research has focused on the local... (Review)
Review
Pulmonary fibrosis is a devastating condition that leads to progressive lung destruction and scarring. Previous mechanistic research has focused on the local fibroproliferative process in the lung. However, emerging evidence suggests that circulating cells of hematopoietic origin play a crucial role in the pathogenesis of this disease.
Topics: Animals; Bleomycin; Fibroblasts; Genetic Predisposition to Disease; Humans; Inflammation; Lung Injury; Models, Biological; Pulmonary Fibrosis; Wound Healing
PubMed: 15286797
DOI: 10.1172/JCI22497 -
Journal of Cellular and Molecular... May 2024Pulmonary fibrosis represents the final alteration seen in a wide variety of lung disorders characterized by increased fibroblast activity and the accumulation of... (Review)
Review
Pulmonary fibrosis represents the final alteration seen in a wide variety of lung disorders characterized by increased fibroblast activity and the accumulation of substantial amounts of extracellular matrix, along with inflammatory damage and the breakdown of tissue architecture. This condition is marked by a significant mortality rate and a lack of effective treatments. The depositing of an excessive quantity of extracellular matrix protein follows the damage to lung capillaries and alveolar epithelial cells, leading to pulmonary fibrosis and irreversible damage to lung function. It has been proposed that the connective tissue growth factor (CTGF) plays a critical role in the advancement of pulmonary fibrosis by enhancing the accumulation of the extracellular matrix and exacerbating fibrosis. In this context, the significance of CTGF in pulmonary fibrosis is examined, and a summary of the development of drugs targeting CTGF for the treatment of pulmonary fibrosis is provided.
Topics: Connective Tissue Growth Factor; Humans; Pulmonary Fibrosis; Animals; Molecular Targeted Therapy; Extracellular Matrix
PubMed: 38774993
DOI: 10.1111/jcmm.18448 -
Clinical and Experimental Medicine Sep 2003Idiopathic pulmonary fibrosis (IPF), synonymous with cryptogenic fibrosing alveolitis (CFA), is a progressive and usually fatal disease of unknown cause characterized by... (Review)
Review
Idiopathic pulmonary fibrosis (IPF), synonymous with cryptogenic fibrosing alveolitis (CFA), is a progressive and usually fatal disease of unknown cause characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Historically, IPF/CFA encompassed a heterogeneous group of different histological and clinical entities arising in an idiopathic setting. Recently, the American Thoracic Society (ATS) and European Respiratory Society (ERS) core committee has redefined diagnostic criteria for both IPF/CFAand idiopathic interstitial pneumonias confining the term IPF/CFA to patients with a histological pattern of usual interstitial pneumonia on lung biopsy. This review attempts to refine the clinico-radiological-pathological features that together define IPF/CFA as it is understood today, and to summarize the rationale of new therapeutic approaches based on the current understanding of the pathogenetic mechanisms.
Topics: Humans; Lung; Lung Diseases, Interstitial; Prognosis; Pulmonary Fibrosis; Radiography, Thoracic; Respiratory Function Tests; Tomography, X-Ray Computed
PubMed: 14598183
DOI: 10.1007/s10238-003-0010-3 -
EBioMedicine Jan 2023
Topics: Humans; Pulmonary Fibrosis; COVID-19; Lung; Respiration
PubMed: 36580850
DOI: 10.1016/j.ebiom.2022.104428 -
European Journal of Pharmaceutical... Apr 2023Pulmonary fibrosis (PF) is a chronic, irreversible lung disease that is typically fatal and characterized by an abnormal fibrotic response. As a result, vast areas of... (Review)
Review
Pulmonary fibrosis (PF) is a chronic, irreversible lung disease that is typically fatal and characterized by an abnormal fibrotic response. As a result, vast areas of the lungs are gradually affected, and gas exchange is impaired, making it one of the world's leading causes of death. This can be attributed to a lack of understanding of the onset and progression of the disease, as well as a poor understanding of the mechanism of adverse responses to various factors, such as exposure to allergens, nanomaterials, environmental pollutants, etc. So far, the most frequently used preclinical evaluation paradigm for PF is still animal testing. Nonetheless, there is an urgent need to understand the factors that induce PF and find novel therapeutic targets for PF in humans. In this regard, robust and realistic in vitro fibrosis models are required to understand the mechanism of adverse responses. Over the years, several in vitro and ex vivo models have been developed with the goal of mimicking the biological barriers of the lung as closely as possible. This review summarizes recent progress towards the development of experimental models suitable for predicting fibrotic responses, with an emphasis on cell culture methods, nanomaterials, and a comparison of results from studies using cells from various species.
Topics: Animals; Humans; Pulmonary Fibrosis; Lung; Nanostructures; Cell Culture Techniques
PubMed: 36652970
DOI: 10.1016/j.ejps.2023.106387