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Pulmonary vasculature
Pulmonary vasculopathy in explanted lungs from patients with interstitial lung disease undergoing lung transplantation
  1. Yaniv Dotan1,
  2. Jeffrey Stewart2,
  3. Andrew Gangemi2,
  4. He Wang3,
  5. Amandeep Aneja3,
  6. Baidarbhi Chakraborty3,
  7. Chandra Dass4,
  8. Huaqing Zhao5,
  9. Nathaniel Marchetti2,
  10. Gilbert D'Alonzo2,
  11. Francis C Cordova2,
  12. Gerard Criner2 and
  13. Albert James Mamary2
  1. 1Pulmonary and Critical Care Medicine, St Luke's University Health Network, Bethlehem, Pennsylvania, USA
  2. 2Department of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, USA
  3. 3Pathology and Laboratory Medicine, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, USA
  4. 4Department of Clinical Radiology, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, USA
  5. 5Department of Clinical Sciences, Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, USA
  1. Correspondence to Dr Yaniv Dotan; yanivdotan{at}gmail.com

Abstract

Background Pulmonary hypertension (PH) causes increased morbidity and mortality in patients with interstitial lung diseases (ILD). Classification schemes, while well-characterised for the vasculopathy of idiopathic PH, have been applied, unchallenged, to ILD-related PH. We evaluated pulmonary arterial histopathology in explanted human lung tissue from patients who were transplanted for advanced fibrotic ILD.

Methods Lung explants from 38 adult patients who underwent lung transplantation were included. Patients were divided into three groups: none, mild/moderate and severe PH by mean pulmonary artery pressure (mPAP) measured at pre lung transplantation right heart catheterisation (RHC). Grading of pulmonary vasculopathy according to Heath and Edwards scheme, and prelung transplantation evaluation data were compared between the groups.

Results 38 patients with fibrotic ILDs were included, the majority (21) with idiopathic pulmonary fibrosis. Of the 38 patients, 18 had severe PH, 13 had mild/moderate PH and 7 had no PH by RHC. 16 of 38 patients had severe pulmonary arterial vasculopathy including vascular occlusion with intimal fibrosis and/or plexiform lesions. There were no correlations between mPAP and lung diffusion with the severity of pulmonary arterial pathological grade (Spearman’s rho=0.14, p=0.34, rho=0.11, p=0.49, respectively).

Conclusions Patients with end stage ILD had severe pulmonary arterial vasculopathy in their explanted lungs irrespective of the presence and/or severity of PH as measured by RHC. These findings suggest that advanced pulmonary arterial vasculopathy is common in patients with advanced fibrotic ILD and may develop prior to the clinical detection of PH by RHC.

  • interstitial fibrosis
  • primary pulmonary hypertension
  • lung transplantation
http://creativecommons.org/licenses/by-nc/4.0/

This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.

https://doi.org/10.1136/bmjresp-2019-000532

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    Footnotes

    • Contributors YD collected the data and is the guarantor of the article, taking responsibility for the integrity of the work as a whole from inception to published article. AG collected the data. JS and GD helped write the manuscript. HW, AA and BC reviewed the pathology studies. HZ conducted the statistical analysis. CD reviewed the imaging studies. NM, FCC, GC and AJM were the transplant pulmonologists for the patients included in the study, conducted the pretransplantation evaluation and treated the patients before and after lung transplantation. AJM was a primary coauthor, responsible for the study concept and helped write the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.

    • Patient consent for publication Not required.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Data availability statement Data are available on reasonable request. All data relevant to the study are included in the article or uploaded as supplementary information. All data relevant to the study are included in the article or uploaded as supplementary information. All data are deidentified participant data. Data are available on reasonable request from the corresponding author, Dr. Yaniv Dotan, at yaniv.dotan@sluhn.org.