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

• Published in: Respiratory medicine case reports Vol. 21; no. C; pp. 74 - 77
• Main Authors: Faure, Morgane, MD, Gomez, Emmanuel, MD, Dorfmüller, Peter, MD, PhD, Mandry, Damien, MD, PhD, Canuet, Matthieu, Kessler, Romain, Chabot, François, Chaouat, Ari, MD, PhD
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 01-01-2017; Elsevier
• Subjects: Bioengineering; Case Report; Computer Science; Emphysema; Human health and pathology; Internal Medicine; Life Sciences; Medical Imaging; Nuclear medicine; Pathology; Pulmonary hypertension; Pulmonary veno-occlusive disease; Pulmonary/Respiratory; Pulmonary veno-occlusive disease; Pathology; Emphysema; Pulmonary hypertension
• ISSN: 2213-0071; 2213-0071
• DOI: 10.1016/j.rmcr.2017.04.001

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.