{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T05:46:29Z","timestamp":1793166389736,"version":"4.5.0"},"reference-count":29,"publisher":"European Respiratory Society (ERS)","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2013,7,30]]},"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:p>A subgroup of patients with idiopathic pulmonary arterial hypertension (IPAH) has severely reduced diffusing capacity of the lung for carbon monoxide (<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>) and poor prognosis. Their characteristics are currently unknown. The aim of this study is to contrast clinical characteristics and treatment responses of IPAH-patients with a severely reduced and more preserved<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>.<\/jats:p><jats:p>Retrospectively, 166 IPAH patients were included and grouped based on a<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>cut-off value of 45% pred (IPAH<jats:sub>&lt;45%<\/jats:sub>and IPAH<jats:sub>\u226545%<\/jats:sub>). Clinical characteristics, treatment responses and survival were compared.<\/jats:p><jats:p>IPAH<jats:sub>&lt;45%<\/jats:sub>were older, more often male, had a more frequent history of coronary disease and a higher tobacco exposure. Forced expiratory volume in 1 s (FEV<jats:sub>1<\/jats:sub>), FEV<jats:sub>1<\/jats:sub>\/forced vital capacity, total lung capacity and alveolar volume values were slightly lower and computed tomography scan abnormalities more prevalent in patients with a low<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>. Age and number of pack years were independently associated with<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>&lt;45% pred. IPAH<jats:sub>&lt;45%<\/jats:sub>showed no different haemodynamic profile, yet worse exercise performance and a worse survival rate, which were both related to age, sex and the presence of coronary disease.<\/jats:p><jats:p>To conclude, a severely reduced<jats:italic>D<\/jats:italic><jats:sub>LCO<\/jats:sub>in IPAH is associated with advanced age and a greater tobacco exposure. These patients have a worse exercise performance despite a similar hemodynamic profile. We confirm the decreased survival in this patient group and now show that this poor outcome is related to age, sex and the presence of coronary disease.<\/jats:p>","DOI":"10.1183\/09031936.00184412","type":"journal-article","created":{"date-parts":[[2013,8,16]],"date-time":"2013-08-16T03:27:31Z","timestamp":1376623651000},"page":"1575-1585","source":"Crossref","is-referenced-by-count":156,"title":["Severely reduced diffusion capacity in idiopathic pulmonary arterial hypertension: patient characteristics and treatment responses"],"prefix":"10.1183","volume":"42","author":[{"given":"Pia","family":"Trip","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esther J.","family":"Nossent","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frances S.","family":"de Man","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inge A.H.","family":"van 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