{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T02:54:05Z","timestamp":1790477645492,"version":"4.1.0"},"reference-count":26,"publisher":"European Respiratory Society (ERS)","issue":"6","license":[{"start":{"date-parts":[[2020,2,27]],"date-time":"2020-02-27T00:00:00Z","timestamp":1582761600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["205188\/Z\/16\/Z"],"award-info":[{"award-number":["205188\/Z\/16\/Z"]}],"id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","award":["FS\/18\/13\/3328"],"award-info":[{"award-number":["FS\/18\/13\/3328"]}],"id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2020,2,16]]},"published-print":{"date-parts":[[2020,6]]},"abstract":"<jats:p>There are limited published data defining survival and treatment response in patients with mild lung disease and\/or reduced gas transfer who fulfil diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH).<\/jats:p>\n                  <jats:p>\n                    Patients diagnosed with IPAH between 2001 and 2019 were identified in the ASPIRE (Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Centre) registry. Using prespecified criteria based on computed tomography (CT) imaging and spirometry, patients with a diagnosis of IPAH and no lung disease were termed IPAH\n                    <jats:sub>no-LD<\/jats:sub>\n                    (n=303), and those with minor\/mild emphysema or fibrosis were described as IPAH\n                    <jats:sub>mild-LD<\/jats:sub>\n                    (n=190).\n                  <\/jats:p>\n                  <jats:p>\n                    Survival was significantly better in IPAH\n                    <jats:sub>no-LD<\/jats:sub>\n                    than in IPAH\n                    <jats:sub>mild-LD<\/jats:sub>\n                    (1- and 5-year survival 95% and 70%\n                    <jats:italic>versus<\/jats:italic>\n                    78% and 22%, respectively; p&lt;0.0001). In the combined group of IPAH\n                    <jats:sub>no-LD<\/jats:sub>\n                    and IPAH\n                    <jats:sub>mild-LD<\/jats:sub>\n                    , independent predictors of higher mortality were increasing age, lower diffusing capacity of the lung for carbon monoxide (\n                    <jats:italic>D<\/jats:italic>\n                    <jats:sub>LCO<\/jats:sub>\n                    ), lower exercise capacity and a diagnosis of IPAH\n                    <jats:sub>mild-LD<\/jats:sub>\n                    (all p&lt;0.05). Exercise capacity and quality of life improved (both p&lt;0.0001) following treatment in patients with IPAH\n                    <jats:sub>no-LD<\/jats:sub>\n                    , but not IPAH\n                    <jats:sub>mild-LD<\/jats:sub>\n                    . A proportion of patients with IPAH\n                    <jats:sub>no-LD<\/jats:sub>\n                    had a\n                    <jats:italic>D<\/jats:italic>\n                    <jats:sub>LCO<\/jats:sub>\n                    &lt;45%; these patients had poorer survival than patients with\n                    <jats:italic>D<\/jats:italic>\n                    <jats:sub>LCO<\/jats:sub>\n                    \u226545%, although they demonstrated improved exercise capacity following treatment.\n                  <\/jats:p>\n                  <jats:p>\n                    The presence of even mild parenchymal lung disease in patients who would be classified as IPAH according to current recommendations has a significant adverse effect on outcomes. This phenotype can be identified using lung function testing and clinical CT reports. Patients with IPAH, no lung disease and severely reduced\n                    <jats:italic>D<\/jats:italic>\n                    <jats:sub>LCO<\/jats:sub>\n                    may represent a further distinct phenotype. These data suggest that randomised controlled trials of targeted therapies in patients with these phenotypes are required.\n                  <\/jats:p>","DOI":"10.1183\/13993003.00041-2020","type":"journal-article","created":{"date-parts":[[2020,2,27]],"date-time":"2020-02-27T08:25:12Z","timestamp":1582791912000},"page":"2000041","update-policy":"https:\/\/doi.org\/10.1183\/ers-crossmark-policy","source":"Crossref","is-referenced-by-count":82,"title":["Mild parenchymal lung disease and\/or low diffusion capacity impacts survival and treatment response in patients diagnosed with idiopathic pulmonary arterial hypertension"],"prefix":"10.1183","volume":"55","author":[{"given":"Robert A.","family":"Lewis","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.A. 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