Open AccessBroad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension
Author(s) -
Sato Takahiro,
Tsujino Ichizo,
Tanino Mishie,
Ohira Hiroshi,
Nishimura Masaharu
Publication year - 2013
Publication title -
respirology case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.304
H-Index - 9
ISSN - 2051-3380
DOI - 10.1002/rcr2.7
Subject(s) - medicine , bosentan , sildenafil , pulmonary hypertension , autopsy , cardiology , lung , pulmonary fibrosis , fibrosis , hemodynamics , pulmonary artery , pathology , receptor , endothelin receptor
A 69‐year‐old man with progressive dyspnea was referred to our hospital in O ct 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 .