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State of the Art in Idiopathic Pulmonary Fibrosis

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A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: closed (30 November 2021) | Viewed by 47471

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Prof. Dr. Malgorzata Wygrecka

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Center for Infection and Genomics of The Lung, Justus-Liebig University Giessen, Aulweg 132, 35392 Giessen, Germany
Interests: lung fibrosis; ECM; coagulation factors; glycosaminoglycans
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Elie El Agha

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Institute for Lung Health (ILH), Justus-Liebig University Giessen, Aulweg 132, 35392 Giessen, Germany
Interests: lung development; stem cells; mesenchymal cells; tissue injury and repair
Special Issues, Collections and Topics in MDPI journals

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Dear Colleagues,

Idiopathic pulmonary fibrosis (IPF) is a lethal disease of unknown etiology, elusive pathogenesis, and very limited therapeutic options. The onset and progression of IPF are influenced by multiple environmental and intrinsic factors, such as exposure to harmful substances, aging and genetic predisposition; however, the magnitude of the contribution of these factors to IPF and the chronological order of downstream pathogenic events remain uncertain. The main hallmarks of IPF are the abnormal activation of lung epithelial cells and the accumulation of fibroblasts/myofibroblasts along with the excessive deposition of extracellular matrix proteins. The aforementioned processes eventually lead to irreversible alveolar scarring, organ malfunction, and death. The incidence and prevalence of IPF are increasing at an alarming rate with the aging population. Recent technological advances and interdisciplinary approaches unmasked the involvment of a broad spectrum of molecular and cellular mediators in the pathogenesis of IPF. Molecules as divere as lipids, RNAs, or peptides, along with a plethora of inflammatory, epithelial, and mesenchymal cell subpopulations turned out to drive maladaptive remodeling to lung tissue. The multifactorial nature of IPF and the lack of robust translational models represent an enormous challenge for the development of successful therapeutic approaches. By critically evaluating the complexity of the disease and the translational value of pre-clinical studies, we would like to provide here a platform for conceptual and technological innovation in the field of IPF and shed light on new therapeutic strategies that may become a part of future treatment options.

This Special Issue encourages the submission of original research articles, reviews or methodological articles dealing with various aspects of IPF research including, but not limited to, the use of in vivo, ex vivo or in vitro models to study lung fibrosis, as well as novel molecular or cellular mediators of lung tissue injury and repair. Studies with clear translational potential are especially welcome.

Prof. Malgorzata Wygrecka
Dr. Elie El Agha
Guest Editors

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Keywords

IPF
ECM
translational models of lung fibrosis
cellular and molecular mediators

Published Papers (11 papers)

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Editorial

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5 pages, 203 KiB

Open AccessEditorial

State of the Art in Idiopathic Pulmonary Fibrosis

by Elie El Agha and Malgorzata Wygrecka

Cells 2022, 11(16), 2487; https://doi.org/10.3390/cells11162487 - 11 Aug 2022

Cited by 1 | Viewed by 1760

Abstract

Idiopathic pulmonary fibrosis (IPF) is a form of usual interstitial pneumonia (UIP), though its origin is unknown [...] Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

Research

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18 pages, 8304 KiB

Open AccessArticle

FGF10 Therapeutic Administration Promotes Mobilization of Injury-Activated Alveolar Progenitors in a Mouse Fibrosis Model

by Yu-Qing Lv, Ge-Fu Cai, Ping-Ping Zeng, Qhaweni Dhlamini, Le-Fu Chen, Jun-Jie Chen, Han-Deng Lyu, Majid Mossahebi-Mohammadi, Negah Ahmadvand, Saverio Bellusci, Xiaokun Li, Chengshui Chen and Jin-San Zhang

Cells 2022, 11(15), 2396; https://doi.org/10.3390/cells11152396 - 3 Aug 2022

Cited by 8 | Viewed by 2433

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease with dire consequences and in urgent need of improved therapies. Compelling evidence indicates that damage or dysfunction of AT2s is of central importance in the development of IPF. We recently identified a novel AT2 subpopulation characterized by low SFTPC expression but that is enriched for PD-L1 in mice. These cells represent quiescent, immature AT2 cells during normal homeostasis and expand upon pneumonectomy (PNX) and were consequently named injury-activated alveolar progenitors (IAAPs). FGF10 is shown to play critical roles in lung development, homeostasis, and injury repair demonstrated in genetically engineered mice. In an effort to bridge the gap between the promising properties of endogenous Fgf10 manipulation and therapeutic reality, we here investigated whether the administration of exogenous recombinant FGF10 protein (rFGF10) can provide preventive and/or therapeutic benefit in a mouse model of bleomycin-induced pulmonary fibrosis with a focus on its impact on IAAP dynamics. C57BL/6 mice and Sftpc^CreERT2/+; tdTomato^flox/+ mice aged 8–10 weeks old were used in this study. To induce the bleomycin (BLM) model, mice were intratracheally (i.t.) instilled with BLM (2 μg/g body weight). BLM injury was induced after a 7-day washout period following tamoxifen induction. A single i.t. injection of rFGF10 (0.05 μg/g body weight) was given on days 0, 7, 14, and 21 after BLM injury. Then, the effects of rFGF10 on BLM-induced fibrosis in lung tissues were assessed by H&E, IHC, Masson’s trichrome staining, hydroxyproline and Western blot assays. Immunofluorescence staining and flow cytometry was used to assess the dynamic behavior of AT2 lineage-labeled Sftpc^Pos (IAAPs and mature AT2) during the course of pulmonary fibrosis. We observed that, depending on the timing of administration, rFGF10 exhibited robust preventive or therapeutic efficacy toward BLM-induced fibrosis based on the evaluation of various pathological parameters. Flow cytometric analysis revealed a dynamic expansion of IAAPs for up to 4 weeks following BLM injury while the number of mature AT2s was drastically reduced. Significantly, rFGF10 administration increased both the peak ratio and the duration of IAAPs expansion relative to EpCAM^Pos cells. Altogether, our results suggest that the administration of rFGF10 exhibits therapeutic potential for IPF most likely by promoting IAAP proliferation and alveolar repair. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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14 pages, 130577 KiB

Open AccessFeature PaperArticle

Transcriptional Profiling of Insulin-like Growth Factor Signaling Components in Embryonic Lung Development and Idiopathic Pulmonary Fibrosis

by Vahid Kheirollahi, Ali Khadim, Georgios Kiliaris, Martina Korfei, Margarida Maria Barroso, Ioannis Alexopoulos, Ana Ivonne Vazquez-Armendariz, Malgorzata Wygrecka, Clemens Ruppert, Andreas Guenther, Werner Seeger, Susanne Herold and Elie El Agha

Cells 2022, 11(12), 1973; https://doi.org/10.3390/cells11121973 - 20 Jun 2022

Cited by 4 | Viewed by 2472

Abstract

Insulin-like growth factor (IGF) signaling controls the development and growth of many organs, including the lung. Loss of function of Igf1 or its receptor Igf1r 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. In this study, we provide a comprehensive transcriptional profile for such signaling components during embryonic lung development in mice, bleomycin-induced pulmonary fibrosis in mice and in human IPF lung explants. During late gestation, we found that Igf1 is upregulated in parallel to Igf1r downregulation in the lung mesenchyme. Lung tissues derived from bleomycin-treated mice and explanted IPF lungs revealed upregulation of IGF1 in parallel to downregulation of IGF1R, in addition to upregulation of several IGF binding proteins (IGFBPs) in lung fibrosis. Finally, treatment of IPF lung fibroblasts with recombinant IGF1 led to myogenic differentiation. Our data serve as a resource for the transcriptional profile of IGF signaling components and warrant further research on the involvement of this pathway in both lung development and pulmonary disease. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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21 pages, 2862 KiB

Open AccessArticle

FK506-Binding Protein 11 Is a Novel Plasma Cell-Specific Antibody Folding Catalyst with Increased Expression in Idiopathic Pulmonary Fibrosis

by Stefan Preisendörfer, Yoshihiro Ishikawa, Elisabeth Hennen, Stephan Winklmeier, Jonas C. Schupp, Larissa Knüppel, Isis E. Fernandez, Leonhard Binzenhöfer, Andrew Flatley, Brenda M. Juan-Guardela, Clemens Ruppert, Andreas Guenther, Marion Frankenberger, Rudolf A. Hatz, Nikolaus Kneidinger, Jürgen Behr, Regina Feederle, Aloys Schepers, Anne Hilgendorff, Naftali Kaminski, Edgar Meinl, Hans Peter Bächinger, Oliver Eickelberg and Claudia A. Staab-Weijnitz Show full author list Hide full author list

Cells 2022, 11(8), 1341; https://doi.org/10.3390/cells11081341 - 14 Apr 2022

Cited by 9 | Viewed by 3513

Abstract

Antibodies are central effectors of the adaptive immune response, widespread used therapeutics, but also potentially disease-causing biomolecules. Antibody folding catalysts in the plasma cell are incompletely defined. Idiopathic pulmonary fibrosis (IPF) is a fatal chronic lung disease with increasingly recognized autoimmune features. We found elevated expression of FK506-binding protein 11 (FKBP11) in IPF lungs where FKBP11 specifically localized to antibody-producing plasma cells. Suggesting a general role in plasma cells, plasma cell-specific FKBP11 expression was equally observed in lymphatic tissues, and in vitro B cell to plasma cell differentiation was accompanied by induction of FKBP11 expression. Recombinant human FKBP11 was able to refold IgG antibody in vitro and inhibited by FK506, strongly supporting a function as antibody peptidyl-prolyl cis-trans isomerase. Induction of ER stress in cell lines demonstrated induction of FKBP11 in the context of the unfolded protein response in an X-box-binding protein 1 (XBP1)-dependent manner. While deficiency of FKBP11 increased susceptibility to ER stress-mediated cell death in an alveolar epithelial cell line, FKBP11 knockdown in an antibody-producing hybridoma cell line neither induced cell death nor decreased expression or secretion of IgG antibody. Similarly, antibody secretion by the same hybridoma cell line was not affected by knockdown of the established antibody peptidyl-prolyl isomerase cyclophilin B. The results are consistent with FKBP11 as a novel XBP1-regulated antibody peptidyl-prolyl cis-trans isomerase and indicate significant redundancy in the ER-resident folding machinery of antibody-producing hybridoma cells. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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22 pages, 4419 KiB

Open AccessArticle

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

by Roxana Maria Wasnick, Irina Shalashova, Jochen Wilhelm, Ali Khadim, Nicolai Schmidt, Holger Hackstein, Andreas Hecker, Konrad Hoetzenecker, Werner Seeger, Saverio Bellusci, Elie El Agha, Clemens Ruppert and Andreas Guenther

Cells 2022, 11(2), 235; https://doi.org/10.3390/cells11020235 - 11 Jan 2022

Cited by 7 | Viewed by 3749

Abstract

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 (Lyso^high and Lyso^low) 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 Lyso^high population expresses all classical AEC2 markers and is drastically diminished in IPF. The Lyso^low 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. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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11 pages, 965 KiB

Open AccessArticle

Differential Discontinuation Profiles between Pirfenidone and Nintedanib in Patients with Idiopathic Pulmonary Fibrosis

by Kazutaka Takehara, Yasuhiko Koga, Yoshimasa Hachisu, Mitsuyoshi Utsugi, Yuri Sawada, Yasuyuki Saito, Seishi Yoshimi, Masakiyo Yatomi, Yuki Shin, Ikuo Wakamatsu, Kazue Umetsu, Shunichi Kouno, Junichi Nakagawa, Noriaki Sunaga, Toshitaka Maeno and Takeshi Hisada

Cells 2022, 11(1), 143; https://doi.org/10.3390/cells11010143 - 2 Jan 2022

Cited by 17 | Viewed by 3046

Abstract

Antifibrotic agents have been widely used in patients with idiopathic pulmonary fibrosis (IPF). Long-term continuation of antifibrotic therapy is required for IPF treatment to prevent disease progression. However, antifibrotic treatment has considerable adverse events, and the continuation of treatment is uncertain in many cases. Therefore, we examined and compared the continuity of treatment between pirfenidone and nintedanib in patients with IPF. We retrospectively enrolled 261 consecutive IPF patients who received antifibrotic treatment from six core facilities in Gunma Prefecture from 2009 to 2018. Among them, 77 patients were excluded if the antifibrotic agent was switched or if the observation period was less than a year. In this study, 134 patients treated with pirfenidone and 50 treated with nintedanib were analyzed. There was no significant difference in patient background, discontinuation rate of antifibrotic treatment over time, and survival rate between the two groups. However, the discontinuation rate due to adverse events within one year of antifibrotic treatment was significantly higher in the nintedanib group than in the pirfenidone group (76% vs. 37%, p < 0.001). Furthermore, the discontinuation rate due to adverse events in nintedanib was higher than that of pirfenidone treatment throughout the observation period (70.6% vs. 31.2%, p = 0.016). The pirfenidone group tended to be discontinued due to acute exacerbation or transfer to another facility. The results of this study suggest that better management of adverse events with nintedanib leads to more continuous treatment that prevents disease progression and acute exacerbations, thus improving prognosis in patients with IPF. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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13 pages, 2080 KiB

Open AccessFeature PaperArticle

Pulmonary Fibroelastotic Remodelling Revisited

by Peter Braubach, Christopher Werlein, Stijn E. Verleden, Isabell Maerzke, Jens Gottlieb, Gregor Warnecke, Sabine Dettmer, Florian Laenger and Danny Jonigk

Cells 2021, 10(6), 1362; https://doi.org/10.3390/cells10061362 - 1 Jun 2021

Cited by 5 | Viewed by 3166

Abstract

Pulmonary fibroelastotic remodelling occurs within a broad spectrum of diseases with vastly divergent outcomes. So far, no comprehensive terminology has been established to adequately address and distinguish histomorphological and clinical entities. We aimed to describe the range of fibroelastotic changes and define stringent histological criteria. Furthermore, we wanted to clarify the corresponding terminology in order to distinguish clinically relevant variants of pulmonary fibroelastotic remodelling. We revisited pulmonary specimens with fibroelastotic remodelling sampled during the last ten years at a large European lung transplant centre. Consensus-based definitions of specific variants of fibroelastotic changes were developed on the basis of well-defined cases and applied. Systematic evaluation was performed in a steps-wise algorithm, first identifying the fulcrum of the respective lesions, and then assessing the morphological changes, their distribution and the features of the adjacent parenchyma. We defined typical alveolar fibro-elastosis as collagenous effacement of the alveolar spaces with accompanying hyper-elastosis of the remodelled and paucicellular alveolar walls, independent of the underlying disease in 45 cases. Clinically, this pattern could be seen in (idiopathic) pleuroparenchymal fibro-elastosis, interstitial lung disease with concomitant alveolar fibro-elastosis, following hematopoietic stem cell and lung transplantation, autoimmune disease, radio-/chemotherapy, and pulmonary apical caps. Novel in-transit and activity stages of fibroelastotic remodelling were identified. For the first time, we present a comprehensive definition of fibroelastotic remodelling, its anatomic distribution, and clinical associations, thereby providing a basis for stringent patient stratification and prediction of outcome. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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Review

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45 pages, 3007 KiB

Open AccessReview

Targeting Histone Deacetylases in Idiopathic Pulmonary Fibrosis: A Future Therapeutic Option

by Martina Korfei, Poornima Mahavadi and Andreas Guenther

Cells 2022, 11(10), 1626; https://doi.org/10.3390/cells11101626 - 12 May 2022

Cited by 24 | Viewed by 4854

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal lung disease with limited therapeutic options, and there is a huge unmet need for new therapies. A growing body of evidence suggests that the histone deacetylase (HDAC) family of transcriptional corepressors has emerged as crucial mediators of IPF pathogenesis. HDACs deacetylate histones and result in chromatin condensation and epigenetic repression of gene transcription. HDACs also catalyse the deacetylation of many non-histone proteins, including transcription factors, thus also leading to changes in the transcriptome and cellular signalling. Increased HDAC expression is associated with cell proliferation, cell growth and anti-apoptosis and is, thus, a salient feature of many cancers. In IPF, induction and abnormal upregulation of Class I and Class II HDAC enzymes in myofibroblast foci, as well as aberrant bronchiolar epithelium, is an eminent observation, whereas type-II alveolar epithelial cells (AECII) of IPF lungs indicate a significant depletion of many HDACs. We thus suggest that the significant imbalance of HDAC activity in IPF lungs, with a “cancer-like” increase in fibroblastic and bronchial cells versus a lack in AECII, promotes and perpetuates fibrosis. This review focuses on the mechanisms by which Class I and Class II HDACs mediate fibrogenesis and on the mechanisms by which various HDAC inhibitors reverse the deregulated epigenetic responses in IPF, supporting HDAC inhibition as promising IPF therapy. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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12 pages, 795 KiB

Open AccessReview

3D In Vitro Models: Novel Insights into Idiopathic Pulmonary Fibrosis Pathophysiology and Drug Screening

by Ana Ivonne Vazquez-Armendariz, Margarida Maria Barroso, Elie El Agha and Susanne Herold

Cells 2022, 11(9), 1526; https://doi.org/10.3390/cells11091526 - 2 May 2022

Cited by 14 | Viewed by 7060

Abstract

Idiopathic pulmonary fibrosis (IPF) is a progressive and often lethal interstitial lung disease of unknown aetiology. IPF is characterised by myofibroblast activation, tissue stiffening, and alveolar epithelium injury. As current IPF treatments fail to halt disease progression or induce regeneration, there is a pressing need for the development of novel therapeutic targets. In this regard, tri-dimensional (3D) models have rapidly emerged as powerful platforms for disease modelling, drug screening and discovery. In this review, we will touch on how 3D in vitro models such as hydrogels, precision-cut lung slices, and, more recently, lung organoids and lung-on-chip devices have been generated and/or modified to reveal distinct cellular and molecular signalling pathways activated during fibrotic processes. Markedly, we will address how these platforms could provide a better understanding of fibrosis pathophysiology and uncover effective treatment strategies for IPF patients. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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27 pages, 3294 KiB

Open AccessReview

Emerging Roles of Airway Epithelial Cells in Idiopathic Pulmonary Fibrosis

by Ashesh Chakraborty, Michal Mastalerz, Meshal Ansari, Herbert B. Schiller and Claudia A. Staab-Weijnitz

Cells 2022, 11(6), 1050; https://doi.org/10.3390/cells11061050 - 19 Mar 2022

Cited by 22 | Viewed by 8638

Abstract

Idiopathic pulmonary fibrosis (IPF) is a fatal disease with incompletely understood aetiology and limited treatment options. Traditionally, IPF was believed to be mainly caused by repetitive injuries to the alveolar epithelium. Several recent lines of evidence, however, suggest that IPF equally involves an aberrant airway epithelial response, which contributes significantly to disease development and progression. In this review, based on recent clinical, high-resolution imaging, genetic, and single-cell RNA sequencing data, we summarize alterations in airway structure, function, and cell type composition in IPF. We furthermore give a comprehensive overview on the genetic and mechanistic evidence pointing towards an essential role of airway epithelial cells in IPF pathogenesis and describe potentially implicated aberrant epithelial signalling pathways and regulation mechanisms in this context. The collected evidence argues for the investigation of possible therapeutic avenues targeting these processes, which thus represent important future directions of research. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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15 pages, 1201 KiB

Open AccessReview

EGFR Signaling in Lung Fibrosis

by Fabian Schramm, Liliana Schaefer and Malgorzata Wygrecka

Cells 2022, 11(6), 986; https://doi.org/10.3390/cells11060986 - 14 Mar 2022

Cited by 19 | Viewed by 5158

Abstract

In this review article, we will first provide a brief overview of the ErbB receptor–ligand system and its importance in developmental and physiological processes. We will then review the literature regarding the role of ErbB receptors and their ligands in the maladaptive remodeling of lung tissue, with special emphasis on idiopathic pulmonary fibrosis (IPF). Here we will focus on the pathways and cellular processes contributing to epithelial–mesenchymal miscommunication seen in this pathology. We will also provide an overview of the in vivo studies addressing the efficacy of different ErbB signaling inhibitors in experimental models of lung injury and highlight how such studies may contribute to our understanding of ErbB biology in the lung. Finally, we will discuss what we learned from clinical applications of the ErbB1 signaling inhibitors in cancer in order to advance clinical trials in IPF. Full article

(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis)

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