Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

Shumar, John; Chandel, Abhimanyu; King, Christopher S.

doi:10.3390/jcm10112285

Cited by 26 publications

(12 citation statements)

References 55 publications

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“…The consensus statement (American Thoracic Society (ATS) and European Respiratory Society (ERS) 2002) on the classification of the ILD described unclassifiable ILD as an interstitial idiopathic lung disease that cannot be categorized based on clinical, radiological and/or pathological findings. Several studies reporting that ILD remains unclassifiable in 10% of all ILD patients and the prognosis is intermediate between fibrosing and non-fibrosing ILD [ 16 , 31 , 38 , 39 ].…”

Section: Pulmonary Vasculitismentioning

confidence: 99%

Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

et al. 2021

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Vasculitides represent a heterogeneous group of immune-mediated disorders, characterized by a systemic inflammatory destructive process of the blood vessels resulting either in ischemia or hemorrhage. The organ involved and vessel size influence the pattern of presentation of the pathology. The lung is commonly involved in systemic vasculitides, with heterogeneous clinical, radiological, and histopathological presentations. Primary vasculitides most commonly associated with lung parenchymal involvement include small-vessel antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Several studies have reported cases of interstitial lung diseases (ILDs) associated with systemic vasculitis, particularly those positive for ANCA associated vasculitis/vasculitidis: AAV. We have selected from our case series different radiological features of pulmonary vasculitis (i.e., solitary or multiple nodules, cavitary lesions, nodules with centrilobular or peribronchial distribution, airspace consolidations, “crazy paving” appearance, interstitial disease), including cases with interstitial lung alterations. Therefore, the aim of this review is to describe the typical clinical manifestations of vasculitides and their main radiologic features (especially AAV).

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Section: Pulmonary Vasculitismentioning

confidence: 99%

Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

et al. 2021

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“…But it seems impossible to timely occurrence reversible and appropriately wound-healing, used to avoid complications ( 6 , 7 ). Despite decades of research on fibrotic diseases, few effective and clinical anti-fibrotic drugs have been discovered yet ( 8 , 9 ). Therefore, it is urgently to explore the pathological process and the underlying mechanism of fibrosis.…”

Section: Introductionmentioning

confidence: 99%

G Protein-Coupled Receptor Kinase 2 as Novel Therapeutic Target in Fibrotic Diseases

Shan

et al. 2022

Front. Immunol.

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G protein-coupled receptor kinase 2 (GRK2), an important subtype of GRKs, specifically phosphorylates agonist-activated G protein-coupled receptors (GPCRs). Besides, current research confirms that it participates in multiple regulation of diverse cells via a non-phosphorylated pathway, including interacting with various non-receptor substrates and binding partners. Fibrosis is a common pathophysiological phenomenon in the repair process of many tissues due to various pathogenic factors such as inflammation, injury, drugs, etc. The characteristics of fibrosis are the activation of fibroblasts leading to myofibroblast proliferation and differentiation, subsequent aggerate excessive deposition of extracellular matrix (ECM). Then, a positive feedback loop is occurred between tissue stiffness caused by ECM and fibroblasts, ultimately resulting in distortion of organ architecture and function. At present, GRK2, which has been described as a multifunctional protein, regulates copious signaling pathways under pathophysiological conditions correlated with fibrotic diseases. Along with GRK2-mediated regulation, there are diverse effects on the growth and apoptosis of different cells, inflammatory response and deposition of ECM, which are essential in organ fibrosis progression. This review is to highlight the relationship between GRK2 and fibrotic diseases based on recent research. It is becoming more convincing that GRK2 could be considered as a potential therapeutic target in many fibrotic diseases.

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“…In idiopathic pulmonary fibrosis (IPF), chronic epithelial injury leads to excessive deposition of rigid extra-cellular matrix and a progressive decrease in lung compliance and gas-exchange surface, causing inevitable and fatal lung failure within 2–5 years after diagnosis [ 1 , 2 , 3 ]. Although new therapies significantly increased the duration and quality of life of IPF patients, a therapeutic regimen that can arrest or, even better, reverse disease progression remains to be discovered [ 4 ]. Partly responsible for this situation is our limited understanding of the cellular states and processes that each of the more than 40 cell types in the lung undergoes, in an active (causative) or reactive manner in homeostatic and injury contexts [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Differential LysoTracker Uptake Defines Two Populations of Distal Epithelial Cells in Idiopathic Pulmonary Fibrosis

Wasnick

Shalashova

Wilhelm

et al. 2022

Cells

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Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal degenerative lung disease of unknown etiology. Although in its final stages it implicates, in a reactive manner, all lung cell types, the initial damage involves the alveolar epithelial compartment, in particular the alveolar epithelial type 2 cells (AEC2s). AEC2s serve dual progenitor and surfactant secreting functions, both of which are deeply impacted in IPF. Thus, we hypothesize that the size of the surfactant processing compartment, as measured by LysoTracker incorporation, allows the identification of different epithelial states in the IPF lung. Flow cytometry analysis of epithelial LysoTracker incorporation delineates two populations (Lysohigh and Lysolow) of AEC2s that behave in a compensatory manner during bleomycin injury and in the donor/IPF lung. Employing flow cytometry and transcriptomic analysis of cells isolated from donor and IPF lungs, we demonstrate that the Lysohigh population expresses all classical AEC2 markers and is drastically diminished in IPF. The Lysolow population, which is increased in proportion in IPF, co-expressed AEC2 and basal cell markers, resembling the phenotype of the previously identified intermediate AEC2 population in the IPF lung. In that regard, we provide an in-depth flow-cytometry characterization of LysoTracker uptake, HTII-280, proSP-C, mature SP-B, NGFR, KRT5, and CD24 expression in human lung epithelial cells. Combining functional analysis with extracellular and intracellular marker expression and transcriptomic analysis, we advance the current understanding of epithelial cell behavior and fate in lung fibrosis.

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Antifibrotic Therapies and Progressive Fibrosing Interstitial Lung Disease (PF-ILD): Building on INBUILD

Cited by 26 publications

References 55 publications

Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

Pulmonary Vasculitides: A Radiological Review Emphasizing Parenchymal HRCT Features

G Protein-Coupled Receptor Kinase 2 as Novel Therapeutic Target in Fibrotic Diseases

Differential LysoTracker Uptake Defines Two Populations of Distal Epithelial Cells in Idiopathic Pulmonary Fibrosis

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