In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans

<p>Abstract</p> <p>Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small...

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Main Authors: Szturmowicz Monika, Kacprzak Aneta, Burakowska Barbara, Kurzyna Marcin, Fijałkowska Anna, Bestry Iwona, Torbicki Adam
Format: Article
Language:English
Published: PAGEPress Publications 2010-12-01
Series:Multidisciplinary Respiratory Medicine
Subjects:
Online Access:http://www.mrmjournal.com/content/5/6/409
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author Szturmowicz Monika
Kacprzak Aneta
Burakowska Barbara
Kurzyna Marcin
Fijałkowska Anna
Bestry Iwona
Torbicki Adam
author_facet Szturmowicz Monika
Kacprzak Aneta
Burakowska Barbara
Kurzyna Marcin
Fijałkowska Anna
Bestry Iwona
Torbicki Adam
author_sort Szturmowicz Monika
collection DOAJ
description <p>Abstract</p> <p>Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease (PVOD). Underdiagnosed PVOD occurs probably in 5-10% of idiopathic pulmonary hypertension (IPAH) and in a substantial proportion of PAH related to connective tissue diseases (mainly in scleroderma). A definite diagnosis of PVOD requires histological examination of lung sample, but surgical lung biopsy in pulmonary hypertension is combined with high risk of bleeding. Thus major interest is focused on a non-invasive diagnostic approach enabling early recognition of PVOD and referral for lung transplantation. The present review is focused on the radiological features suggestive of PVOD-like vasculopathy in PAH.</p>
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spelling doaj.art-56878984eb2e4dfe9eaad7be745982472024-03-02T03:57:12ZengPAGEPress PublicationsMultidisciplinary Respiratory Medicine2049-69582010-12-015640941610.1186/2049-6958-5-6-409In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scansSzturmowicz MonikaKacprzak AnetaBurakowska BarbaraKurzyna MarcinFijałkowska AnnaBestry IwonaTorbicki Adam<p>Abstract</p> <p>Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease (PVOD). Underdiagnosed PVOD occurs probably in 5-10% of idiopathic pulmonary hypertension (IPAH) and in a substantial proportion of PAH related to connective tissue diseases (mainly in scleroderma). A definite diagnosis of PVOD requires histological examination of lung sample, but surgical lung biopsy in pulmonary hypertension is combined with high risk of bleeding. Thus major interest is focused on a non-invasive diagnostic approach enabling early recognition of PVOD and referral for lung transplantation. The present review is focused on the radiological features suggestive of PVOD-like vasculopathy in PAH.</p>http://www.mrmjournal.com/content/5/6/409CT scanidiopathic pulmonary hypertensionmosaic lung attenuationpulmonary veno-occlusive disease
spellingShingle Szturmowicz Monika
Kacprzak Aneta
Burakowska Barbara
Kurzyna Marcin
Fijałkowska Anna
Bestry Iwona
Torbicki Adam
In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans
Multidisciplinary Respiratory Medicine
CT scan
idiopathic pulmonary hypertension
mosaic lung attenuation
pulmonary veno-occlusive disease
title In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans
title_full In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans
title_fullStr In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans
title_full_unstemmed In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans
title_short In search of markers of treatment failure and poor prognosis in IPAH - the value of mosaic lung attenuation pattern on thin-section CT scans
title_sort in search of markers of treatment failure and poor prognosis in ipah the value of mosaic lung attenuation pattern on thin section ct scans
topic CT scan
idiopathic pulmonary hypertension
mosaic lung attenuation
pulmonary veno-occlusive disease
url http://www.mrmjournal.com/content/5/6/409
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