{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T07:43:17Z","timestamp":1791186197084,"version":"4.1.0"},"reference-count":38,"publisher":"European Respiratory Society (ERS)","issue":"6","funder":[{"name":"Grant from the French National Institute for Health and Medical Research"},{"name":"Legs Poix Grant"}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2016,8,29]]},"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:p>\n                    The impact of bone morphogenetic protein receptor 2 (\n                    <jats:italic>BMPR2<\/jats:italic>\n                    ) gene mutations on vascular remodelling in pulmonary arterial hypertension (PAH) is unknown. We sought to identify a histological profile of\n                    <jats:italic>BMPR2<\/jats:italic>\n                    mutation carriers.\n                  <\/jats:p>\n                  <jats:p>Clinical data and lung histology from 44 PAH patients were subjected to systematic analysis and morphometry.<\/jats:p>\n                  <jats:p>\n                    Bronchial artery hypertrophy\/dilatation and bronchial angiogenesis, as well as muscular remodelling of septal veins were significantly increased in PAH lungs carrying\n                    <jats:italic>BMPR2<\/jats:italic>\n                    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 (&gt;50\u2005mL) was significantly more frequent in\n                    <jats:italic>BMPR2<\/jats:italic>\n                    mutation carriers. 43.5% of\n                    <jats:italic>BMPR2<\/jats:italic>\n                    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.\n                  <\/jats:p>\n                  <jats:p>\n                    Our analysis provides evidence for the involvement of the pulmonary systemic circulation in\n                    <jats:italic>BMPR2<\/jats:italic>\n                    mutation-related PAH. We show that\n                    <jats:italic>BMPR2<\/jats:italic>\n                    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\n                    <jats:italic>BMPR2<\/jats:italic>\n                    mutation.\n                  <\/jats:p>","DOI":"10.1183\/13993003.00464-2016","type":"journal-article","created":{"date-parts":[[2016,11,3]],"date-time":"2016-11-03T21:10:50Z","timestamp":1478207450000},"page":"1668-1681","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":97,"title":["<i>BMPR2<\/i>\n                    mutation status influences bronchial vascular changes in pulmonary arterial hypertension"],"prefix":"10.1183","volume":"48","author":[{"given":"Maria-Rosa","family":"Ghigna","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8545-4452","authenticated-orcid":false,"given":"Christophe","family":"Guignabert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Montani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Girerd","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Ja\u00efs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Savale","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Herv\u00e9","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"Thomas de Montpr\u00e9ville","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olaf","family":"Mercier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Sitbon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florent","family":"Soubrier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elie","family":"Fadel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G\u00e9rald","family":"Simonneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Dorfm\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"81","published-online":{"date-parts":[[2016,11,3]]},"reference":[{"key":"2024102100145698000_48.6.1668.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.029"},{"key":"2024102100145698000_48.6.1668.2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.025"},{"key":"2024102100145698000_48.6.1668.3","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01032-2015"},{"key":"2024102100145698000_48.6.1668.4","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.023"},{"key":"2024102100145698000_48.6.1668.5","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200510-1668OC"},{"key":"2024102100145698000_48.6.1668.6","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201205-0886OC"},{"key":"2024102100145698000_48.6.1668.7","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2013.10.035"},{"key":"2024102100145698000_48.6.1668.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200108023450503"},{"key":"2024102100145698000_48.6.1668.9","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.108.800938"},{"key":"2024102100145698000_48.6.1668.10","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200712-1807OC"},{"key":"2024102100145698000_48.6.1668.11","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(15)00544-5"},{"key":"2024102100145698000_48.6.1668.12","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.105.601930"},{"key":"2024102100145698000_48.6.1668.13","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0078132"},{"key":"2024102100145698000_48.6.1668.14","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.104.488486"},{"key":"2024102100145698000_48.6.1668.15","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201201-0164OC"},{"key":"2024102100145698000_48.6.1668.16","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.18.4.533"},{"key":"2024102100145698000_48.6.1668.17","unstructured":"Pietra G . 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