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

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.

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Lung histology of samples obtained after lung transplantation. As typically seen in pulmonary veno-occlusive disease (PVOD), small pulmonary veins and venules are partially or completely occluded by paucicellular fibrous thickening of the intimal layer (a). In addition, pulmonary arteries (b, bottom left) display eccentric wall-thickening. Also, focal broadening of the alveolar walls with multiplication of alveolar capillaries is observed (b, inset center), corresponding to pulmonary capillary hemangiomatosis, a feature that is typically observed in PVOD, these patchy foci are frequently associated with muscularized microvessels (b, inset top). Note the areas with emphysematous loss of alveoli (b, top and bottom).
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fig2: Lung histology of samples obtained after lung transplantation. As typically seen in pulmonary veno-occlusive disease (PVOD), small pulmonary veins and venules are partially or completely occluded by paucicellular fibrous thickening of the intimal layer (a). In addition, pulmonary arteries (b, bottom left) display eccentric wall-thickening. Also, focal broadening of the alveolar walls with multiplication of alveolar capillaries is observed (b, inset center), corresponding to pulmonary capillary hemangiomatosis, a feature that is typically observed in PVOD, these patchy foci are frequently associated with muscularized microvessels (b, inset top). Note the areas with emphysematous loss of alveoli (b, top and bottom).

Mentions: Due to an overall insufficient response to medical treatment, the patient was placed on a lung-transplant waiting list, which was successfully performed seven months later. Histology of the explanted lungs showed pre- and post-capillary lesions involving pulmonary arteries, pulmonary veins, and pre-septal venules, leading to partial occlusion of the lumen (Fig. 2). Vein and venular remodeling showed paucicellular intimal fibrosis. There was patchy distribution and abnormal multiplication of capillaries along the alveolar walls, which corresponded to pulmonary capillary hemangiomatosis. Small arteries and arterioles showed eccentric intimal fibrosis. This histological examination was consistent with PVOD. However, uncommonly for PVOD, emphysematous loss of alveoli and moderate interstitial and bronchiolar lymphocytic infiltrates were observed, sometimes in close association with pulmonary vascular lesions.


A diffuse lung emphysema, severe pulmonary hypertension and lack of airflow limitation
Lung histology of samples obtained after lung transplantation. As typically seen in pulmonary veno-occlusive disease (PVOD), small pulmonary veins and venules are partially or completely occluded by paucicellular fibrous thickening of the intimal layer (a). In addition, pulmonary arteries (b, bottom left) display eccentric wall-thickening. Also, focal broadening of the alveolar walls with multiplication of alveolar capillaries is observed (b, inset center), corresponding to pulmonary capillary hemangiomatosis, a feature that is typically observed in PVOD, these patchy foci are frequently associated with muscularized microvessels (b, inset top). Note the areas with emphysematous loss of alveoli (b, top and bottom).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

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

fig2: Lung histology of samples obtained after lung transplantation. As typically seen in pulmonary veno-occlusive disease (PVOD), small pulmonary veins and venules are partially or completely occluded by paucicellular fibrous thickening of the intimal layer (a). In addition, pulmonary arteries (b, bottom left) display eccentric wall-thickening. Also, focal broadening of the alveolar walls with multiplication of alveolar capillaries is observed (b, inset center), corresponding to pulmonary capillary hemangiomatosis, a feature that is typically observed in PVOD, these patchy foci are frequently associated with muscularized microvessels (b, inset top). Note the areas with emphysematous loss of alveoli (b, top and bottom).
Mentions: Due to an overall insufficient response to medical treatment, the patient was placed on a lung-transplant waiting list, which was successfully performed seven months later. Histology of the explanted lungs showed pre- and post-capillary lesions involving pulmonary arteries, pulmonary veins, and pre-septal venules, leading to partial occlusion of the lumen (Fig. 2). Vein and venular remodeling showed paucicellular intimal fibrosis. There was patchy distribution and abnormal multiplication of capillaries along the alveolar walls, which corresponded to pulmonary capillary hemangiomatosis. Small arteries and arterioles showed eccentric intimal fibrosis. This histological examination was consistent with PVOD. However, uncommonly for PVOD, emphysematous loss of alveoli and moderate interstitial and bronchiolar lymphocytic infiltrates were observed, sometimes in close association with pulmonary vascular lesions.

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