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Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension

Frédéric Perros, Marc Humbert, Peter Dorfmüller
European Respiratory Review 2021 30: 210161; DOI: 10.1183/16000617.0161-2021
Frédéric Perros
1Université Paris-Saclay, School of Medicine, Le Kremlin Bicêtre, France
2INSERM UMR S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
3Paris-Porto Pulmonary Hypertension Collaborative Laboratory (3PH), INSERM, Le Kremlin-Bicêtre, France
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Marc Humbert
1Université Paris-Saclay, School of Medicine, Le Kremlin Bicêtre, France
2INSERM UMR S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
4Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
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Peter Dorfmüller
5Institut für Pathologie, Universitätklinikum Giessen und Marburg, Giessen, Germany
6Deutsches Zentrum für Lungenforschung (DZL), Giessen, Germany
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  • For correspondence: peter.dorfmuller@patho.med.uni-giessen.de
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Figures

  • FIGURE 1
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    FIGURE 1

    Pulmonary arterial hypertension characteristics.

  • FIGURE 2
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    FIGURE 2

    Inflammatory mechanisms involved in pulmonary arterial hypertension (PAH) pathogenesis.

  • FIGURE 3
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    FIGURE 3

    The two types of mechanism that underlie pulmonary arterial hypertension pathogenesis.

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    FIGURE 4

    Structural alterations as a cause of inflammation. Disturbed blood flow and stiffness-induced inflammation. ECM: extra-cellular matrix.

  • FIGURE 5
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    FIGURE 5

    Hypoxia-induced inflammation. BDNF: brain-derived neurotrophic factor; PAEC: pulmonary arterial endothelial cell; PASMC: pulmonary arterial smooth muscle cell; TSLP: thymic stromal lymphopoietin; VEGF: vascular endothelial growth factor.

  • FIGURE 6
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    FIGURE 6

    Pattern recognition receptor (PRR)-induced inflammation in pulmonary arterial hypertension. DAMP: damage-associated molecular pattern; HMGB1: high mobility group box-1; LPS: lipopolysaccharide; PAMP: pathogen-associated molecular pattern; RAGE: receptor for advanced glycation end products; TLR4: toll-like receptor 4.

  • FIGURE 7
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    FIGURE 7

    Transcription factors linking vascular remodelling and inflammation. ERG: ETS-related gene; FLI-1: Friend leukaemia integration 1 transcription factor.

  • FIGURE 8
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    FIGURE 8

    Inflammation as a cause of structural alterations: autoimmunity in pulmonary arterial hypertension (PAH). CCL20: chemokine (C-C motif) ligand 20; CXCL13: chemokine ligand 13; FOXP: Forkhead box P; IL-17: interleukin-17; MHC: major histocompatibility complex; MoDC: monocyte-derived dendritic cell; NK: natural killer cell; NKT: natural killer T-cell; Th17: T-helper 17; tLT: tertiary lymphoid tissue.

  • FIGURE 9
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    FIGURE 9

    Heritable pulmonary arterial hypertension: predisposing mutations involved in immune regulation. BMPR2: bone morphogenetic protein receptor type 2; ER: endoplasmic reticulum; GCN2: general control nonderepressible 2; IL: interleukin; KCNK3: potassium two pore domain channel subfamily K member 3; PVOD: pulmonary veno-occlusive disease; TGF-β: transforming growth factor-β.

  • FIGURE 10
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    FIGURE 10

    Controversies and consequences of pulmonary arterial hypertension therapy.

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    FIGURE 11

    Key points to take away.

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Vol 30 Issue 162 Table of Contents
European Respiratory Review: 30 (162)
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Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
Frédéric Perros, Marc Humbert, Peter Dorfmüller
European Respiratory Review Dec 2021, 30 (162) 210161; DOI: 10.1183/16000617.0161-2021

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Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension
Frédéric Perros, Marc Humbert, Peter Dorfmüller
European Respiratory Review Dec 2021, 30 (162) 210161; DOI: 10.1183/16000617.0161-2021
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  • Article
    • Abstract
    • Abstract
    • Introduction
    • The smouldering fire hypothesis
    • The conflagration hypothesis
    • Targeting inflammation and and dysregulated immunity in PAH
    • Conclusion
    • Acknowledgements
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

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  • Pulmonary vascular disease
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