Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension

Respirology Case Reports

Takahiro Sato²  Ichizo Tsujino²  Mishie Tanino¹  Hiroshi Ohira² 

[1] Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine, Hokkaido, Japan;First Department of Medicine, Hokkaido University Hospital, Hokkaido, Japan

关键词: phosphodiesterase 5;    endothelin;    prostaglandin I2;   

DOI  :  10.1002/rcr2.7

来源: Wiley

【 摘 要 】

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.

【 授权许可】

CC BY-NC   
© 2013 The Authors. Respirology Case Reports published by John Wiley & Sons Ltd on behalf of The Asian Pacific Society of Respirology.

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