Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline

American Journal of Respiratory and Critical Care Medicine, Journal Year: 2022, Volume and Issue: 205(9), P. e18 - e47

Published: April 29, 2022

Background: This American Thoracic Society, European Respiratory Japanese and Asociación Latinoamericana de Tórax guideline updates prior idiopathic pulmonary fibrosis (IPF) guidelines addresses the progression of in patients with interstitial lung diseases (ILDs) other than IPF. Methods: A committee was composed multidisciplinary experts ILD, methodologists, patient representatives. 1) Update IPF: Radiological histopathological criteria for IPF were updated by consensus. Questions about transbronchial cryobiopsy, genomic classifier testing, antacid medication, antireflux surgery informed systematic reviews answered evidence-based recommendations using Grading Recommendations, Assessment, Development Evaluation (GRADE) approach. 2) Progressive (PPF): PPF defined, then radiological physiological determined pirfenidone nintedanib GRADE Results:1) conditional recommendation made to regard cryobiopsy as an acceptable alternative surgical biopsy centers appropriate expertise. No or against testing. Conditional medication treatment PPF: defined at least two three (worsening symptoms, progression, progression) occurring within past year no explanation a ILD nintedanib, additional research into recommended. Conclusions: The this are intended provide basis rational, decisions clinicians.

Language: Английский

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Dictionary learning for integrative, multimodal and scalable single-cell analysis

Nature Biotechnology, Journal Year: 2023, Volume and Issue: 42(2), P. 293 - 304

Published: May 25, 2023

Language: Английский

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Cross-tissue organization of the fibroblast lineage

Nature, Journal Year: 2021, Volume and Issue: 593(7860), P. 575 - 579

Published: May 12, 2021

Language: Английский

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Circuits between infected macrophages and T cells in SARS-CoV-2 pneumonia

Nature, Journal Year: 2021, Volume and Issue: 590(7847), P. 635 - 641

Published: Jan. 11, 2021

Language: Английский

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Alveolar regeneration through a Krt8+ transitional stem cell state that persists in human lung fibrosis

Nature Communications, Journal Year: 2020, Volume and Issue: 11(1)

Published: July 16, 2020

Abstract The cell type specific sequences of transcriptional programs during lung regeneration have remained elusive. Using time-series single RNA-seq the bleomycin injury model, we resolved dynamics for 28 types. Trajectory modeling together with lineage tracing revealed that airway and alveolar stem cells converge on a unique Krt8 + transitional state regeneration. These squamous morphology, feature p53 NFkB activation display features cellular senescence. Krt8+ appears in several independent models persists human fibrosis, creating distinct cell–cell communication network mesenchyme macrophages repair. We generated model gene regulatory leading to their terminal differentiation type-1 cells. propose perturbed molecular checkpoints way can cause aberrant persistence regenerative intermediate states.

Language: Английский

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Inflammatory Signals Induce AT2 Cell-Derived Damage-Associated Transient Progenitors that Mediate Alveolar Regeneration

Cell stem cell, Journal Year: 2020, Volume and Issue: 27(3), P. 366 - 382.e7

Published: Aug. 3, 2020

Tissue regeneration is a multi-step process mediated by diverse cellular hierarchies and states that are also implicated in tissue dysfunction pathogenesis. Here we leveraged single-cell RNA sequencing combination with vivo lineage tracing organoid models to finely map the trajectories of alveolar-lineage cells during injury repair lung regeneration. We identified distinct AT2-lineage population, damage-associated transient progenitors (DATPs), arises alveolar found interstitial macrophage-derived IL-1β primes subset AT2 expressing Il1r1 for conversion into DATPs via HIF1α-mediated glycolysis pathway, which required mature AT1 cell differentiation. Importantly, chronic inflammation prevents differentiation, leading aberrant accumulation impaired Together, this stepwise mapping fate transitions shows how an inflammatory niche controls controlling stem behavior.

Language: Английский

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Pathogenic Mechanisms Underlying Idiopathic Pulmonary Fibrosis

Annual Review of Pathology Mechanisms of Disease, Journal Year: 2021, Volume and Issue: 17(1), P. 515 - 546

Published: Nov. 23, 2021

The pathogenesis of idiopathic pulmonary fibrosis (IPF) involves a complex interplay cell types and signaling pathways. Recurrent alveolar epithelial (AEC) injury may occur in the context predisposing factors (e.g., genetic, environmental, epigenetic, immunologic, gerontologic), leading to metabolic dysfunction, senescence, aberrant activation, dysregulated repair. interacts with mesenchymal, immune, endothelial cells via multiple mechanisms trigger fibroblast myofibroblast activation. Recent single-cell RNA sequencing studies IPF lungs support model. These have uncovered novel type AEC characteristics an basal cell, which disrupt normal repair propagate profibrotic phenotype. Here, we review bioinformatics tools as strategies discover pathways disease, cell-specific mechanisms, cell-cell interactions that niche.

Language: Английский

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Persistence of a regeneration-associated, transitional alveolar epithelial cell state in pulmonary fibrosis

Nature Cell Biology, Journal Year: 2020, Volume and Issue: 22(8), P. 934 - 946

Published: July 13, 2020

Language: Английский

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Idiopathic pulmonary fibrosis: Disease mechanisms and drug development

Pharmacology & Therapeutics, Journal Year: 2020, Volume and Issue: 222, P. 107798 - 107798

Published: Dec. 24, 2020

Language: Английский

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Transfer learning enables predictions in network biology

Nature, Journal Year: 2023, Volume and Issue: 618(7965), P. 616 - 624

Published: May 31, 2023

Language: Английский

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