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Sato T, Tsujino I, Tanino M, et al. Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension. Respirol Case Rep. 2013;1(1):10-3doi: 10.1002/rcr2.7.
Sato, T., Tsujino, I., Tanino, M., Ohira, H., & Nishimura, M. (2013). Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension. Respirology case reports, 1(1), 10-3. https://doi.org/10.1002/rcr2.7
Sato, Takahiro, et al. "Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension." Respirology case reports vol. 1,1 (2013): 10-3. doi: https://doi.org/10.1002/rcr2.7
Sato T, Tsujino I, Tanino M, Ohira H, Nishimura M. Broad and heterogeneous vasculopathy in pulmonary fibrosis and emphysema with pulmonary hypertension. Respirol Case Rep. 2013 Sep;1(1):10-3. doi: 10.1002/rcr2.7. PMID: 25473528; PMCID: PMC4184639.
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Respirol Case Rep. 2013 Sep;1(1):10-3. doi: 10.1002/rcr2.7.

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

Respirology case reports

Takahiro Sato, Ichizo Tsujino, Mishie Tanino, Hiroshi Ohira, Masaharu Nishimura

Affiliations

  1. First Department of Medicine, Hokkaido University Hospital Hokkaido, Japan.
  2. Laboratory of Cancer Research, Department of Pathology, Hokkaido University Graduate School of Medicine Hokkaido, Japan.
  3. Division of Respiratory Medicine, Department of Internal Medicine, Hokkaido University Graduate School of Medicine Hokkaido, Japan.

PMID: 25473528 PMCID: PMC4184639 DOI: 10.1002/rcr2.7

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.

Keywords: endothelin; phosphodiesterase 5; prostaglandin I2

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