TY - JOUR
T1 - Pulmonary Hypertension Associated with Idiopathic Pulmonary Fibrosis
T2 - Current and Future Perspectives
AU - Collum, Scott D.
AU - Amione-Guerra, Javier
AU - Cruz-Solbes, Ana S.
AU - Difrancesco, Amara
AU - Hernandez, Adriana M.
AU - Hanmandlu, Ankit
AU - Youker, Keith
AU - Guha, Ashrith
AU - Karmouty-Quintana, Harry
N1 - Publisher Copyright:
© 2017 Scott D. Collum et al.
PY - 2017
Y1 - 2017
N2 - Pulmonary hypertension (PH) is commonly present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO). PH has been identified to be present in as much as 40% of patients with COPD or IPF and it is considered as one of the principal predictors of mortality in patients with COPD or IPF. However, despite the prevalence and fatal consequences of PH in the setting of chronic lung diseases, there are limited therapies available for patients with Group III PH, with lung transplantation remaining as the most viable option. This highlights our need to enhance our understanding of the molecular mechanisms that lead to the development of Group III PH. In this review we have chosen to focus on the current understating of PH in IPF, we will revisit the main mediators that have been shown to play a role in the development of the disease. We will also discuss the experimental models available to study PH associated with lung fibrosis and address the role of the right ventricle in IPF. Finally we will summarize the current available treatment options for Group III PH outside of lung transplantation.
AB - Pulmonary hypertension (PH) is commonly present in patients with chronic lung diseases such as Chronic Obstructive Pulmonary Disease (COPD) or Idiopathic Pulmonary Fibrosis (IPF) where it is classified as Group III PH by the World Health Organization (WHO). PH has been identified to be present in as much as 40% of patients with COPD or IPF and it is considered as one of the principal predictors of mortality in patients with COPD or IPF. However, despite the prevalence and fatal consequences of PH in the setting of chronic lung diseases, there are limited therapies available for patients with Group III PH, with lung transplantation remaining as the most viable option. This highlights our need to enhance our understanding of the molecular mechanisms that lead to the development of Group III PH. In this review we have chosen to focus on the current understating of PH in IPF, we will revisit the main mediators that have been shown to play a role in the development of the disease. We will also discuss the experimental models available to study PH associated with lung fibrosis and address the role of the right ventricle in IPF. Finally we will summarize the current available treatment options for Group III PH outside of lung transplantation.
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U2 - 10.1155/2017/1430350
DO - 10.1155/2017/1430350
M3 - Review article
C2 - 28286407
AN - SCOPUS:85014163044
SN - 1198-2241
VL - 2017
JO - Canadian Respiratory Journal
JF - Canadian Respiratory Journal
M1 - 1430350
ER -