title: Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension.
abstract: A 69-year-old man with progressive dyspnea was referred to our hospital in Oct 2010. The patient was clinically diagnosed with combined pulmonary fibrosis and emphysema (CPFE) and pulmonary hypertension (PH). Sildenafil and bosentan were used for the treatment of progressive PH, and dyspnea and pulmonary hemodynamics improved at 3 months follow-up. However, the patient died of respiratory failure 1 year later. Autopsy identified marked intimal and medial thickening of the pulmonary arteries/arterioles, and modest but broad fibrous obstruction of the veins/venules and capillary multiplication. Also, immunohistochemical study showed positive staining for the target proteins of the PH-specific vasodilators, sildenafil and bosentan, on the diseased vessels. The present autopsy report is the first to pathologically document the diseased pulmonary vasculature and how PH-vasodilators can ameliorate pulmonary hemodynamics in a patient with CPFE and PH.
authors.affiliation: First Department of Medicine, Hokkaido University Hospital Hokkaido, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Sato
authors.affiliation: First Department of Medicine, Hokkaido University Hospital Hokkaido, Japan.
authors.forename: Ichizo
authors.initials: I
authors.lastname: Tsujino
authors.affiliation: Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine Hokkaido, Japan.
authors.forename: Mishie
authors.initials: M
authors.lastname: Tanino
authors.affiliation: First Department of Medicine, Hokkaido University Hospital Hokkaido, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ohira
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, Hokkaido University Graduate School of Medicine Hokkaido, Japan.
authors.forename: Masaharu
authors.initials: M
authors.lastname: Nishimura
keywords: endothelin; phosphodiesterase 5; prostaglandin I2