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Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy

The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5–15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant conditions. Therefore, any signi...

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Autores principales: Kacprzak, Aneta, Tomkowski, Witold, Szturmowicz, Monika
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10378268/
https://www.ncbi.nlm.nih.gov/pubmed/37510098
http://dx.doi.org/10.3390/diagnostics13142354
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author Kacprzak, Aneta
Tomkowski, Witold
Szturmowicz, Monika
author_facet Kacprzak, Aneta
Tomkowski, Witold
Szturmowicz, Monika
author_sort Kacprzak, Aneta
collection PubMed
description The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5–15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant conditions. Therefore, any significant PH occurring in the setting of ILD requires a proper differential workup. PH increases morbidity and mortality in ILDs. The pathomechanisms underlying PH due to ILD (PH-ILD) are not fully known, and there is no straightforward correlation between the presence or severity of PH-ILD and the severity of ILD. Severe PH in mild ILD without other explanatory causes constitutes a dilemma of differentiating between PH due to ILD and pulmonary arterial hypertension coexisting with ILDs. The heterogeneity and poor prognosis of patients with ILDs coexisting with PH necessitate an individualised approach to the management of this condition. This review presents recent advances in understanding and treatment options in PH-ILD. It also addresses practical issues, such as when to suspect and how to screen for PH in ILD, what are the indications for right heart catheterisation, and how to approach an individual ILD patient to determine the dominant PH cause and apply adequate management.
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spelling pubmed-103782682023-07-29 Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy Kacprzak, Aneta Tomkowski, Witold Szturmowicz, Monika Diagnostics (Basel) Review The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5–15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant conditions. Therefore, any significant PH occurring in the setting of ILD requires a proper differential workup. PH increases morbidity and mortality in ILDs. The pathomechanisms underlying PH due to ILD (PH-ILD) are not fully known, and there is no straightforward correlation between the presence or severity of PH-ILD and the severity of ILD. Severe PH in mild ILD without other explanatory causes constitutes a dilemma of differentiating between PH due to ILD and pulmonary arterial hypertension coexisting with ILDs. The heterogeneity and poor prognosis of patients with ILDs coexisting with PH necessitate an individualised approach to the management of this condition. This review presents recent advances in understanding and treatment options in PH-ILD. It also addresses practical issues, such as when to suspect and how to screen for PH in ILD, what are the indications for right heart catheterisation, and how to approach an individual ILD patient to determine the dominant PH cause and apply adequate management. MDPI 2023-07-13 /pmc/articles/PMC10378268/ /pubmed/37510098 http://dx.doi.org/10.3390/diagnostics13142354 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Kacprzak, Aneta
Tomkowski, Witold
Szturmowicz, Monika
Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
title Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
title_full Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
title_fullStr Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
title_full_unstemmed Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
title_short Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy
title_sort pulmonary hypertension in the course of interstitial lung diseases—a personalised approach is needed to identify a dominant cause and provide an effective therapy
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10378268/
https://www.ncbi.nlm.nih.gov/pubmed/37510098
http://dx.doi.org/10.3390/diagnostics13142354
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