What is it about?

What’s it about? Pulmonary arterial hypertension (PAH) is a rare and serious disease caused by changes in the vessels that carry blood from the heart to the lungs. The arteries and arterioles become thick and stiff, which means that blood cannot pass through as easily as it would do normally and so the blood pressure in these vessels increases. There are many chemical and biological processes or pathways in the body that can be disrupted in PAH and may contribute to the development of the disease. Currently available treatments for PAH target three of these biological pathways. However, there are many other pathways that also contribute to development of PAH. This article discusses research into some of the less well-known processes. Why is it important? It is important to look at the reasons why a disease occurs so that appropriate therapies can be developed. Investigation of the less well-known pathways will improve our understanding of why PAH occurs and ultimately help to increase the number of therapies available to patients.

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This page is a summary of: Pathobiology of pulmonary arterial hypertension: understanding the roads less travelled, European Respiratory Review, December 2017, European Respiratory Society (ERS),
DOI: 10.1183/16000617.0093-2017.
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