Pulmonary hypertension in interstitial lung disease

J. Behr, Jay H Ryu

Research output: Contribution to journalArticle

170 Citations (Scopus)

Abstract

In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs. Copyright

Original languageEnglish (US)
Pages (from-to)1357-1367
Number of pages11
JournalEuropean Respiratory Journal
Volume31
Issue number6
DOIs
StatePublished - Jun 2008

Fingerprint

Interstitial Lung Diseases
Pulmonary Hypertension
Lung Transplantation
Brain Natriuretic Peptide
Exercise
Oxygen
Therapeutics
Delayed Diagnosis
Cardiac Catheterization
Echocardiography
Comorbidity
Biomarkers
Clinical Trials
Hormones
Lung

Keywords

  • Connective tissue disease
  • Interstitial lung disease
  • Pulmonary fibrosis
  • Pulmonary hypertension
  • Sarcoidosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Medicine(all)

Cite this

Pulmonary hypertension in interstitial lung disease. / Behr, J.; Ryu, Jay H.

In: European Respiratory Journal, Vol. 31, No. 6, 06.2008, p. 1357-1367.

Research output: Contribution to journalArticle

@article{6201f9b07e7a4be2a949c4f9feea3bba,
title = "Pulmonary hypertension in interstitial lung disease",
abstract = "In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40{\%}) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs. Copyright",
keywords = "Connective tissue disease, Interstitial lung disease, Pulmonary fibrosis, Pulmonary hypertension, Sarcoidosis",
author = "J. Behr and Ryu, {Jay H}",
year = "2008",
month = "6",
doi = "10.1183/09031936.00171307",
language = "English (US)",
volume = "31",
pages = "1357--1367",
journal = "European Respiratory Journal",
issn = "0903-1936",
publisher = "European Respiratory Society",
number = "6",

}

TY - JOUR

T1 - Pulmonary hypertension in interstitial lung disease

AU - Behr, J.

AU - Ryu, Jay H

PY - 2008/6

Y1 - 2008/6

N2 - In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs. Copyright

AB - In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs. Copyright

KW - Connective tissue disease

KW - Interstitial lung disease

KW - Pulmonary fibrosis

KW - Pulmonary hypertension

KW - Sarcoidosis

UR - http://www.scopus.com/inward/record.url?scp=49649117743&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=49649117743&partnerID=8YFLogxK

U2 - 10.1183/09031936.00171307

DO - 10.1183/09031936.00171307

M3 - Article

VL - 31

SP - 1357

EP - 1367

JO - European Respiratory Journal

JF - European Respiratory Journal

SN - 0903-1936

IS - 6

ER -