Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Foris, V; Brcic, L; Douschan, P; Kovacs, G; Stacher-Priehse, E; Olschewski, H.
Advanced interstitial lung fibrosis with emphysema and pulmonary hypertension with no evidence for interstitial lung disease on high resolution CT.
Pulm Circ. 2019; 9(3): 2045894019832214
Doi: 10.1177/2045894019832214
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- Führende Autor*innen der Med Uni Graz
- Foris Vasile
- Olschewski Horst
- Co-Autor*innen der Med Uni Graz
- Brcic Luka
- Douschan Philipp
- Kovacs Gabor
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- Abstract:
- 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.
- Find related publications in this database (Keywords)
- DLCO
- idiopathic pulmonary arterial hypertension
- high-resolution lung CT
- lung histology