Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT
The diagnosis of idiopathic pulmonary arterial hypertension (iPAH) is complex and, besides invasive hemodynamic evaluation, includes several diagnostic steps to exclude any underlying diseases. The role of a decreased diffusion capacity of the lung for carbon monoxide (DLCO) is a matter of discussio...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2019-07-01
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| Series: | Pulmonary Circulation |
| Online Access: | https://doi.org/10.1177/2045894019832214 |
| _version_ | 1818245990074810368 |
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| author | Vasile Foris Luka Brcic Philipp Douschan Gabor Kovacs Elvira Stacher-Priehse Horst Olschewski |
| author_facet | Vasile Foris Luka Brcic Philipp Douschan Gabor Kovacs Elvira Stacher-Priehse Horst Olschewski |
| author_sort | Vasile Foris |
| collection | DOAJ |
| description | The diagnosis of idiopathic pulmonary arterial hypertension (iPAH) is complex and, besides invasive hemodynamic evaluation, includes several diagnostic steps to exclude any underlying diseases. The role of a decreased diffusion capacity of the lung for carbon monoxide (DLCO) is a matter of discussion. Here, we present a 76-year-old man with a smoking history of 30 pack-years who was diagnosed with iPAH after chronic thromboembolic pulmonary hypertension was excluded based on a negative perfusion scan, an underlying heart disease was excluded based on echocardiography and right heart catheterization, and a significant lung disease was excluded based on lung function test (FVC = 101% predicted, FEV1 = 104% predicted, FEV1/FVC = 77, TLC = 97% predicted) and thin-slice computed tomography (CT) scan. Just DLCO was reduced to 40% predicted, suggesting a possible structural lung disease. Postmortem examination demonstrated severe interstitial lung fibrosis combined with microscopic emphysema. This indicates that both CT imaging and pulmonary function test may be insensitive to a diffuse peripheral combined pattern of fibrosis and emphysema and that DLCO may be the only sensitive marker of this significant lung pathology. |
| first_indexed | 2024-12-12T14:41:41Z |
| format | Article |
| id | doaj.art-0787d368392e43ee9453939778d7ac78 |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-12-12T14:41:41Z |
| publishDate | 2019-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-0787d368392e43ee9453939778d7ac782022-12-22T00:21:13ZengWileyPulmonary Circulation2045-89402019-07-01910.1177/2045894019832214Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CTVasile Foris0Luka Brcic1Philipp Douschan2Gabor Kovacs3Elvira Stacher-Priehse4Horst Olschewski5Ludwig Boltzmann Institute for Lung Vascular Research, Graz, AustriaMedical University of Graz, Institute of Pathology, Graz, AustriaLudwig Boltzmann Institute for Lung Vascular Research, Graz, AustriaLudwig Boltzmann Institute for Lung Vascular Research, Graz, AustriaMedical University of Graz, Institute of Pathology, Graz, AustriaLudwig Boltzmann Institute for Lung Vascular Research, Graz, AustriaThe diagnosis of idiopathic pulmonary arterial hypertension (iPAH) is complex and, besides invasive hemodynamic evaluation, includes several diagnostic steps to exclude any underlying diseases. The role of a decreased diffusion capacity of the lung for carbon monoxide (DLCO) is a matter of discussion. Here, we present a 76-year-old man with a smoking history of 30 pack-years who was diagnosed with iPAH after chronic thromboembolic pulmonary hypertension was excluded based on a negative perfusion scan, an underlying heart disease was excluded based on echocardiography and right heart catheterization, and a significant lung disease was excluded based on lung function test (FVC = 101% predicted, FEV1 = 104% predicted, FEV1/FVC = 77, TLC = 97% predicted) and thin-slice computed tomography (CT) scan. Just DLCO was reduced to 40% predicted, suggesting a possible structural lung disease. Postmortem examination demonstrated severe interstitial lung fibrosis combined with microscopic emphysema. This indicates that both CT imaging and pulmonary function test may be insensitive to a diffuse peripheral combined pattern of fibrosis and emphysema and that DLCO may be the only sensitive marker of this significant lung pathology.https://doi.org/10.1177/2045894019832214 |
| spellingShingle | Vasile Foris Luka Brcic Philipp Douschan Gabor Kovacs Elvira Stacher-Priehse Horst Olschewski Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT Pulmonary Circulation |
| title | Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT |
| title_full | Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT |
| title_fullStr | Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT |
| title_full_unstemmed | Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT |
| title_short | Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT |
| title_sort | advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution ct |
| url | https://doi.org/10.1177/2045894019832214 |
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