PT - JOURNAL ARTICLE AU - Hemnes, Anna R. AU - Humbert, Marc 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