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A diffuse lung emphysema, severe pulmonary hypertension and lack of airflow limitation

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ABSTRACT

Pulmonary veno-occlusive disease is characterized by remodeling of pulmonary arteries, capillaries and venules. We report a case of diffuse lung emphysema and pulmonary veno-occlusive disease with the characteristic of having no airflow limitation. A very low diffusing capacity for carbon monoxide and results of high-resolution computed tomography of the chest suggested pulmonary veno-occlusive disease. The diagnosis was confirmed on histological analysis after lung transplantation. The combination of results of the computed tomography of the chest and the histological analysis suggested a relationship between diffuse lung emphysema and remodeling of pulmonary vessels. A distinctive pattern of mild-to-moderate airflow limitation in patients with chronic obstructive pulmonary disease and severe pulmonary hypertension has been described. This observation of the combination of diffuse emphysema, pulmonary veno-occlusive disease and no airflow limitation supports further pathophysiological studies on severe pulmonary hypertension in chronic obstructive pulmonary disease.

No MeSH data available.


Related in: MedlinePlus

High-resolution computed tomography of the chest with major diffuse centrilobular emphysema, characteristic signs of pulmonary veno-occlusive disease and no evidence of pulmonary fibrosis: patchy ground-glass opacities, and few septal lines; and mediastinal and hilar lymphadenopathies. Note the presence of significant development of arteries in the mediastinum from the systemic circulation.
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fig1: High-resolution computed tomography of the chest with major diffuse centrilobular emphysema, characteristic signs of pulmonary veno-occlusive disease and no evidence of pulmonary fibrosis: patchy ground-glass opacities, and few septal lines; and mediastinal and hilar lymphadenopathies. Note the presence of significant development of arteries in the mediastinum from the systemic circulation.

Mentions: A ventilation/perfusion lung scan excluded a chronic thromboembolic PH. Pulmonary function tests showed an increase in all pulmonary volumes including forced expiratory volume in 1 s (FEV1). Forced vital capacity, FEV1 and total lung capacity were 144 of the predicted value (% pred.), 130% pred. and 133% pred., respectively. Diffusing capacity for carbon monoxide was very low, 27 of % pred. High-resolution CT of the chest showed major diffuse centrilobular emphysema, patchy ground-glass opacities, few septal lines, and mediastinal and hilar lymphadenopathies (Fig. 1). Arterial blood gases assessed under stable condition revealed severe hypoxemia (PaO2 46 mm Hg) and hypocapnia (PaCO2 23 mm Hg). Serum level of alpha1-antitrypsin was in the normal range (130 mg/mL).


A diffuse lung emphysema, severe pulmonary hypertension and lack of airflow limitation
High-resolution computed tomography of the chest with major diffuse centrilobular emphysema, characteristic signs of pulmonary veno-occlusive disease and no evidence of pulmonary fibrosis: patchy ground-glass opacities, and few septal lines; and mediastinal and hilar lymphadenopathies. Note the presence of significant development of arteries in the mediastinum from the systemic circulation.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5384413&req=5

fig1: High-resolution computed tomography of the chest with major diffuse centrilobular emphysema, characteristic signs of pulmonary veno-occlusive disease and no evidence of pulmonary fibrosis: patchy ground-glass opacities, and few septal lines; and mediastinal and hilar lymphadenopathies. Note the presence of significant development of arteries in the mediastinum from the systemic circulation.
Mentions: A ventilation/perfusion lung scan excluded a chronic thromboembolic PH. Pulmonary function tests showed an increase in all pulmonary volumes including forced expiratory volume in 1 s (FEV1). Forced vital capacity, FEV1 and total lung capacity were 144 of the predicted value (% pred.), 130% pred. and 133% pred., respectively. Diffusing capacity for carbon monoxide was very low, 27 of % pred. High-resolution CT of the chest showed major diffuse centrilobular emphysema, patchy ground-glass opacities, few septal lines, and mediastinal and hilar lymphadenopathies (Fig. 1). Arterial blood gases assessed under stable condition revealed severe hypoxemia (PaO2 46 mm Hg) and hypocapnia (PaCO2 23 mm Hg). Serum level of alpha1-antitrypsin was in the normal range (130 mg/mL).

View Article: PubMed Central - PubMed

ABSTRACT

Pulmonary veno-occlusive disease is characterized by remodeling of pulmonary arteries, capillaries and venules. We report a case of diffuse lung emphysema and pulmonary veno-occlusive disease with the characteristic of having no airflow limitation. A very low diffusing capacity for carbon monoxide and results of high-resolution computed tomography of the chest suggested pulmonary veno-occlusive disease. The diagnosis was confirmed on histological analysis after lung transplantation. The combination of results of the computed tomography of the chest and the histological analysis suggested a relationship between diffuse lung emphysema and remodeling of pulmonary vessels. A distinctive pattern of mild-to-moderate airflow limitation in patients with chronic obstructive pulmonary disease and severe pulmonary hypertension has been described. This observation of the combination of diffuse emphysema, pulmonary veno-occlusive disease and no airflow limitation supports further pathophysiological studies on severe pulmonary hypertension in chronic obstructive pulmonary disease.

No MeSH data available.


Related in: MedlinePlus