Cargando…

The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease

Pulmonary hypertension (PH) in patients with Systemic Sclerosis (SSc) may stem from a variety of underlying causes, thus making a correct diagnosis and management difficult. The main challenges lie in the distinction between pulmonary arterial hypertension (PAH, group 1) and PH due to interstitial l...

Descripción completa

Detalles Bibliográficos
Autores principales: Zanatta, Elisabetta, Marra, Martina Perazzolo, Famoso, Giulia, Balestro, Elisabetta, Giraudo, Chiara, Calabrese, Fiorella, Rea, Federico, Doria, Andrea
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9504220/
https://www.ncbi.nlm.nih.gov/pubmed/36145263
http://dx.doi.org/10.3390/ph15091042
_version_ 1784796161415053312
author Zanatta, Elisabetta
Marra, Martina Perazzolo
Famoso, Giulia
Balestro, Elisabetta
Giraudo, Chiara
Calabrese, Fiorella
Rea, Federico
Doria, Andrea
author_facet Zanatta, Elisabetta
Marra, Martina Perazzolo
Famoso, Giulia
Balestro, Elisabetta
Giraudo, Chiara
Calabrese, Fiorella
Rea, Federico
Doria, Andrea
author_sort Zanatta, Elisabetta
collection PubMed
description Pulmonary hypertension (PH) in patients with Systemic Sclerosis (SSc) may stem from a variety of underlying causes, thus making a correct diagnosis and management difficult. The main challenges lie in the distinction between pulmonary arterial hypertension (PAH, group 1) and PH due to interstitial lung disease (PH-ILD, group 3) in patients with concomitant lung fibrosis — a very common occurrence in SSc. A consensus among experts remains elusive. Some studies have suggested that among SSc patients with PH, those with an ILD extension > 20% at high-resolution computed tomography (HRCT) should be considered as affected by PH-ILD, whereas other Authors have found that a wide proportion of these patients exhibit features of both PAH and group 3 PH-ILD. We report the case of a 46-year-old male SSc patient with a stable and extensive ILD (>20%) who developed a histologically documented pulmonary vasculopathy typical of PAH and received PAH-specific treatment as bridge to transplant. Moreover, we documented PH disease course by right heart catheterization (RHC), with and without specific vasodilator therapies, which are essential in PAH but not indicated and/or harmful in PH-ILD.
format Online
Article
Text
id pubmed-9504220
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-95042202022-09-24 The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease Zanatta, Elisabetta Marra, Martina Perazzolo Famoso, Giulia Balestro, Elisabetta Giraudo, Chiara Calabrese, Fiorella Rea, Federico Doria, Andrea Pharmaceuticals (Basel) Case Report Pulmonary hypertension (PH) in patients with Systemic Sclerosis (SSc) may stem from a variety of underlying causes, thus making a correct diagnosis and management difficult. The main challenges lie in the distinction between pulmonary arterial hypertension (PAH, group 1) and PH due to interstitial lung disease (PH-ILD, group 3) in patients with concomitant lung fibrosis — a very common occurrence in SSc. A consensus among experts remains elusive. Some studies have suggested that among SSc patients with PH, those with an ILD extension > 20% at high-resolution computed tomography (HRCT) should be considered as affected by PH-ILD, whereas other Authors have found that a wide proportion of these patients exhibit features of both PAH and group 3 PH-ILD. We report the case of a 46-year-old male SSc patient with a stable and extensive ILD (>20%) who developed a histologically documented pulmonary vasculopathy typical of PAH and received PAH-specific treatment as bridge to transplant. Moreover, we documented PH disease course by right heart catheterization (RHC), with and without specific vasodilator therapies, which are essential in PAH but not indicated and/or harmful in PH-ILD. MDPI 2022-08-24 /pmc/articles/PMC9504220/ /pubmed/36145263 http://dx.doi.org/10.3390/ph15091042 Text en © 2022 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 Case Report
Zanatta, Elisabetta
Marra, Martina Perazzolo
Famoso, Giulia
Balestro, Elisabetta
Giraudo, Chiara
Calabrese, Fiorella
Rea, Federico
Doria, Andrea
The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease
title The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease
title_full The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease
title_fullStr The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease
title_full_unstemmed The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease
title_short The Challenge of Diagnosing and Managing Pulmonary Arterial Hypertension in Systemic Sclerosis with Interstitial Lung Disease
title_sort challenge of diagnosing and managing pulmonary arterial hypertension in systemic sclerosis with interstitial lung disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9504220/
https://www.ncbi.nlm.nih.gov/pubmed/36145263
http://dx.doi.org/10.3390/ph15091042
work_keys_str_mv AT zanattaelisabetta thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT marramartinaperazzolo thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT famosogiulia thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT balestroelisabetta thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT giraudochiara thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT calabresefiorella thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT reafederico thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT doriaandrea thechallengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT zanattaelisabetta challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT marramartinaperazzolo challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT famosogiulia challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT balestroelisabetta challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT giraudochiara challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT calabresefiorella challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT reafederico challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease
AT doriaandrea challengeofdiagnosingandmanagingpulmonaryarterialhypertensioninsystemicsclerosiswithinterstitiallungdisease