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Pulmonary hypertensive vasculopathy in parenchymal lung diseases and/or hypoxia

Maria Rosa Ghigna, Wolter J. Mooi, Katrien Grünberg
European Respiratory Review 2017 26: 170003; DOI: 10.1183/16000617.0003-2017
Maria Rosa Ghigna
1Service d'Anatomie et de Cytologie Pathologiques, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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  • For correspondence: mr.ghigna@ccml.fr
Wolter J. Mooi
2Dept of Pathology, VU University Medical Center, Amsterdam, The Netherlands
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Katrien Grünberg
3Dept of Pathology, Radboud UMC, Nijmegen, The Netherlands
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  • FIGURE 1
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    FIGURE 1

    Small pulmonary arteries exhibiting irregular intimal and medial thickening within emphysematous lung parenchyma. Haematoxylin–eosin–saffron staining. Scale bar=100 µm.

  • FIGURE 2
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    FIGURE 2

    a) Actin immunostaining of a remodelled small pulmonary artery. The neointima is thickened by the affluence of cells phenotypically similar to smooth muscle cells (SMC-like cells), expressing actin. b) Desmin immunostaining of the same remodelled pulmonary artery. The neointimal SMC-like cells do not express desmin, unlike medial smooth muscle cells. Both images courtesy of P. Dorfmüller, Hôpital Marie Lannelongue, Le Plessis Robinson, France. Scale bars=100 µm.

  • FIGURE 3
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    FIGURE 3

    a) Small pulmonary arteries within a fibrotic area (usual interstitial pneumonia lung). The narrowing of the vascular lumen is the result of both intimal fibrosis and medial smooth muscle increase. Haematoxylin–eosin–saffron staining. Scale bar=250 μm. b) Remodelled small pulmonary arteries outside fibrosis in usual interstitial pneumonia lung. Haematoxylin–eosin–saffron staining. Scale bar=200 μm.

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  • TABLE 1

    Classification of pulmonary hypertension, group 3

    1 Pulmonary arterial hypertension
    1′ Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
    1′′ Persistent pulmonary hypertension of the newborn
    2 Pulmonary hypertension owing to left heart disease
    3 Pulmonary hypertension owing to lung diseases and/or hypoxia
    3.1 Chronic obstructive pulmonary disease
    3.2 Interstitial lung disease
    3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
    3.4 Sleep disordered breathing
    3.5 Alveolar hypoventilation disorders
    3.6 Chronic exposure to high altitude
    3.7 Developmental abnormalities
    4 Chronic thromboembolic pulmonary hypertension
    5 Pulmonary hypertension with unclear multifactorial mechanisms

    Data from [1].

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    Vol 26 Issue 144 Table of Contents
    European Respiratory Review: 26 (144)
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    Pulmonary hypertensive vasculopathy in parenchymal lung diseases and/or hypoxia
    Maria Rosa Ghigna, Wolter J. Mooi, Katrien Grünberg
    European Respiratory Review Jun 2017, 26 (144) 170003; DOI: 10.1183/16000617.0003-2017

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    Pulmonary hypertensive vasculopathy in parenchymal lung diseases and/or hypoxia
    Maria Rosa Ghigna, Wolter J. Mooi, Katrien Grünberg
    European Respiratory Review Jun 2017, 26 (144) 170003; DOI: 10.1183/16000617.0003-2017
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    • Article
      • Abstract
      • Abstract
      • Introduction
      • “Pure” hypoxic PH: clinical data
      • PH in COPD and interstitial lung disease: clinical data
      • Pathogenesis of PH in COPD
      • Pathogenesis of PH in IPF
      • Conclusion
      • Footnotes
      • References
    • Figures & Data
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    Subjects

    • Mechanisms of lung disease
    • Pulmonary vascular disease
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    Series

    • New antibiotics for Gram-negative pneumonia
    • Severe community-acquired pneumonia
    • Pulmonary aspergillosis: diagnosis and treatment
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    Pathology for the Clinician

    • Unclassifiable interstitial lung disease
    • Lung ageing and COPD
    • Differential diagnosis of granulomatous lung disease
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