Ponticos, Markella;
Lee, Michael;
Holmes, Alan;
Tuder, Rubin;
(2024)
Pathogenesis of Pulmonary Arterial Hypertension.
In: Allanore, Yannick and Varga, John and Denton, Christopher P and Kuwana, Masataka and Chung, Lorinda and Shah, Ami, (eds.)
Scleroderma: From Pathogenesis to Comprehensive Management (Third edition).
(pp. 421-444).
Springer: Cham, Switzerland.
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Abstract
The pathogenic mechanisms by which pulmonary arterial hypertension (PAH) develops and progresses are complex and not fully elucidated. Considerable heterogeneity in disease pathological features, some of which are unique and some common among the PAH spectrum of diseases, including idiopathic and scleroderma-associated PAH, adds further to this complexity. Our understanding so far has significantly progressed and has underpinned some of the successful therapeutics that are being used to treat PAH resulting in marked improvement in survival. In this revised chapter, we explore the biological processes and mechanistic insights that have advanced the field, the genetic and epigenetic components, the role of inflammation and metabolism, the cellular and molecular pathways, and their importance in the context of disease heterogeneity.
| Type: | Book chapter |
|---|---|
| Title: | Pathogenesis of Pulmonary Arterial Hypertension |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1007/978-3-031-40658-4_27 |
| Publisher version: | https://doi.org/10.1007/978-3-031-40658-4_27 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10193698 |
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