RT Journal Article SR Electronic T1 BMPR2 mutation status influences bronchial vascular changes in pulmonary arterial hypertension JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 1668 OP 1681 DO 10.1183/13993003.00464-2016 VO 48 IS 6 A1 Maria-Rosa Ghigna A1 Christophe Guignabert A1 David Montani A1 Barbara Girerd A1 Xavier Jaïs A1 Laurent Savale A1 Philippe Hervé A1 Vincent Thomas de Montpréville A1 Olaf Mercier A1 Olivier Sitbon A1 Florent Soubrier A1 Elie Fadel A1 Gérald Simonneau A1 Marc Humbert A1 Peter Dorfmüller YR 2016 UL http://erj.ersjournals.com/content/48/6/1668.abstract AB The impact of bone morphogenetic protein receptor 2 (BMPR2) gene mutations on vascular remodelling in pulmonary arterial hypertension (PAH) is unknown. We sought to identify a histological profile of BMPR2 mutation carriers.Clinical data and lung histology from 44 PAH patients were subjected to systematic analysis and morphometry.Bronchial artery hypertrophy/dilatation and bronchial angiogenesis, as well as muscular remodelling of septal veins were significantly increased in PAH lungs carrying BMPR2 mutations. We found that patients displaying increased bronchial artery remodelling and bronchial microvessel density, irrespective of the mutation status, were more likely to suffer from severe haemoptysis. History of substantial haemoptysis (>50 mL) was significantly more frequent in BMPR2 mutation carriers. 43.5% of BMPR2 mutation carriers, as opposed to 9.5% of noncarriers, displayed singular large fibrovascular lesions, which appear to be closely related to the systemic lung vasculature.Our analysis provides evidence for the involvement of the pulmonary systemic circulation in BMPR2 mutation-related PAH. We show that BMPR2 mutation carriers are more prone to haemoptysis and that haemoptysis is closely correlated to bronchial arterial remodelling and angiogenesis; in turn, pronounced changes in the systemic vasculature correlate with increased pulmonary venous remodelling, creating a distinctive profile in PAH patients harbouring a BMPR2 mutation.A distinctive pattern in hereditary PAH relating exuberant development of systemic lung vasculature to haemoptysis http://ow.ly/Iz2f303N1oN