Extract
Pulmonary arterial hypertension (PAH) is a rare disease of the pulmonary vasculature characterised by progressive remodelling and narrowing of the pulmonary arteries due to pathological abnormalities of fibroblasts, inflammatory cells, endothelial cells and smooth muscle cells [1]. Decades of clinical, translational and bench research have advanced our understanding of the underlying mechanisms contributing to the development of PAH. However, despite considerable heterogeneity of this disease, current diagnostic and treatment guidelines remain largely standardised. Aside from cohorts with long-term response to calcium channel blockers, individualised treatment options targeted at the various phenotypes of PAH have not been identified.
Abstract
New, sophisticated tools are being used to advance our knowledge of PAH and facilitate individualised treatment based on phenotypes http://ow.ly/qP6h30ocllp
Footnotes
Conflict of interest: E.M. Beck has nothing to disclose.
Conflict of interest: N.D. Hatton has nothing to disclose.
Conflict of interest: J.J. Ryan has nothing to disclose.
- Received March 19, 2019.
- Accepted March 20, 2019.
- Copyright ©ERS 2019