PT  - JOURNAL ARTICLE
AU  - Anna R. Hemnes
AU  - Marc Humbert
TI  - Pathobiology of pulmonary arterial hypertension: understanding the roads less travelled
AID  - 10.1183/16000617.0093-2017
DP  - 2017 Dec 31
TA  - European Respiratory Review
PG  - 170093
VI  - 26
IP  - 146
4099  - http://err.ersjournals.com/content/26/146/170093.short
4100  - http://err.ersjournals.com/content/26/146/170093.full
SO  - EUROPEAN RESPIRATORY REVIEW2017 Dec 31; 26
AB  - The pathobiology of pulmonary arterial hypertension (PAH) is complex and incompletely understood. Although three pathogenic pathways have been relatively well characterised, it is widely accepted that dysfunction in a multitude of other cellular processes is likely to play a critical role in driving the development of PAH. Currently available therapies, which all target one of the three well-characterised pathways, provide significant benefits for patients; however, PAH remains a progressive and ultimately fatal disease. The development of drugs to target alternative pathogenic pathways is, therefore, an attractive proposition and one that may complement existing treatment regimens to improve outcomes for patients. Considerable research has been undertaken to identify the role of the less well-understood pathways and in this review we will highlight some of the key discoveries and the potential for utility as therapeutic targets.Dysfunction in a multitude of cellular processes plays a critical role in driving the development of PAH http://ow.ly/mGsY30haDXB