Pulmonary Hypertension in Rare Parenchymal Lung Diseases

Pulmonary hypertension related to chronic lung disease, mainly represented by COPD and idiopathic pulmonary fibrosis, is associated with a worse outcome when compared with patients only affected by parenchymal lung disease. At present, no therapies are available to reverse or slow down the pathological process of this condition and most of the clinical trials conducted to date have had no clinically significant impact. Nevertheless, the importance of chronic lung diseases is always more widely recognised and, along with its increasing incidence, associated pulmonary hypertension is also expected to be growing in frequency and as a health burden worldwide. Therefore, it is desirable to develop useful and reliable tools to obtain an early diagnosis and to monitor and follow-up this condition, while new insights in the therapeutic approach are explored.

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Pulmonary Hypertension in Parenchymal Lung Disease

Pulmonary Medicine ◽

10.1155/2012/684781 ◽

2012 ◽

Vol 2012 ◽

pp. 1-14 ◽

Cited By ~ 3

Author(s):

Iraklis Tsangaris ◽

Georgios Tsaknis ◽

Anastasia Anthi ◽

Stylianos E. Orfanos

Keyword(s):

Pulmonary Hypertension ◽

Lung Diseases ◽

Interstitial Lung Diseases ◽

Idiopathic Pulmonary Arterial Hypertension ◽

Chronic Obstructive ◽

Obstructive Pulmonary Disease ◽

Diffuse Parenchymal Lung Diseases ◽

Pulmonary Arterial ◽

Parenchymal Lung Disease ◽

Parenchymal Lung

Idiopathic pulmonary arterial hypertension (IPAH) has been extensively investigated, although it represents a less common form of the pulmonary hypertension (PH) family, as shown by international registries. Interestingly, in types of PH that are encountered in parenchymal lung diseases such as interstitial lung diseases (ILDs), chronic obstructive pulmonary disease (COPD), and many other diffuse parenchymal lung diseases, some of which are very common, the available data is limited. In this paper, we try to browse in the latest available data regarding the occurrence, pathogenesis, and treatment of PH in chronic parenchymal lung diseases.

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Management of Pulmonary Hypertension Due to Chronic Lung Disease

Methodist DeBakey Cardiovascular Journal ◽

10.14797/zkut3813 ◽

2021 ◽

Vol 17 (2) ◽

pp. 124-133

Author(s):

Jordan Sugarman ◽

Jason Weatherald

Keyword(s):

Pulmonary Hypertension ◽

Lung Disease ◽

Chronic Lung Disease ◽

Lung Diseases ◽

Chronic Obstructive Lung Disease ◽

Pulmonary Vasculature ◽

Chronic Obstructive ◽

Group 3 ◽

Group 5 ◽

Parenchymal Lung

Pulmonary hypertension (PH) is a known complication of chronic parenchymal lung diseases, including chronic obstructive lung disease, interstitial lung diseases, and more rare parenchymal lung diseases. Together, these diseases encompass two of the five clinical classifications of PH: group 3 (chronic lung disease [CLD] and/or hypoxia) and group 5 (unclear and/or multifactorial mechanisms). The principal management strategy in PH associated with CLD is optimization of the underlying lung disease. There has been increasing interest in therapies that treat pulmonary arterial hypertension (group 1, PAH), and although some studies have explored the use of these oral PAH-targeted therapies to treat PH associated with CLD , there is currently no evidence to support their routine use; in fact, some studies suggest harm. Inhaled therapies that target the pulmonary vasculature may avoid certain problems observed with oral PAH therapies. Recent studies suggest a promising role for inhaled PAH therapies in group 3 PH, but this requires further study. The objective of this article is to review the current treatment strategies for group 3 and group 5 PH.

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