Phenotyping of idiopathic pulmonary arterial hypertension

• Verfasst von: Höper, Marius [VerfasserIn]
• Verfasst von: Dwivedi, Krit [VerfasserIn]
• Verfasst von: Pausch, Christine [VerfasserIn]
• Verfasst von: Lewis, Robert A [VerfasserIn]
• Verfasst von: Olsson, Karen Maria [VerfasserIn]
• Verfasst von: Huscher, Doerte [VerfasserIn]
• Verfasst von: Pittrow, David [VerfasserIn]
• Verfasst von: Grünig, Ekkehard [VerfasserIn]
• Verfasst von: Stähler, Gerd [VerfasserIn]
• Verfasst von: Vizza, Carmine Dario [VerfasserIn]
• Verfasst von: Gall, Henning [VerfasserIn]
• Verfasst von: Distler, Oliver [VerfasserIn]
• Verfasst von: Opitz, Christian F. [VerfasserIn]
• Verfasst von: Gibbs, John Simon R [VerfasserIn]
• Verfasst von: Delcroix, Marion [VerfasserIn]
• Verfasst von: Park, Da-Hee [VerfasserIn]
• Verfasst von: Ghofrani, Hossein Ardeschir [VerfasserIn]
• Verfasst von: Ewert, Ralf [VerfasserIn]
• Verfasst von: Kaemmerer, Harald [VerfasserIn]
• Verfasst von: Kabitz, Hans-Joachim [VerfasserIn]
• Verfasst von: Skowasch, Dirk [VerfasserIn]
• Verfasst von: Behr, Jürgen [VerfasserIn]
• Verfasst von: Milger-Kneidinger, Katrin [VerfasserIn]
• Verfasst von: Lange, Tobias J [VerfasserIn]
• Verfasst von: Wilkens, Heinrike [VerfasserIn]
• Verfasst von: Seyfarth, Hans-Jürgen [VerfasserIn]
• Verfasst von: Held, Matthias [VerfasserIn]
• Verfasst von: Dumitrescu, Daniel [VerfasserIn]
• Verfasst von: Tsangaris, Iraklis [VerfasserIn]
• Verfasst von: Vonk-Noordegraaf, Anton [VerfasserIn]
• Verfasst von: Ulrich Somaini, Silvia [VerfasserIn]
• Verfasst von: Klose, Hans [VerfasserIn]
• Verfasst von: Claussen, Martin [VerfasserIn]
• Verfasst von: Eisenmann, Stephan [VerfasserIn]
• Verfasst von: Schmidt, Kai Helge [VerfasserIn]
• Verfasst von: Swift, Andrew J [VerfasserIn]
• Verfasst von: Thompson, Alfred A Roger [VerfasserIn]
• Verfasst von: Elliot, Charlie A [VerfasserIn]
• Verfasst von: Rosenkranz, Stephan [VerfasserIn]
• Verfasst von: Condliffe, Robin [VerfasserIn]
• Verfasst von: Kiely, David G [VerfasserIn]
• Verfasst von: Halank, Michael [VerfasserIn]
• Titel: Phenotyping of idiopathic pulmonary arterial hypertension
• Titelzusatz: a registry analysis
• Verf.angabe: Marius M Hoeper, Krit Dwivedi, Christine Pausch, Robert A Lewis, Karen M Olsson, Doerte Huscher, David Pittrow, Ekkehard Grünig, Gerd Staehler, Carmine Dario Vizza, Henning Gall, Oliver Distler, Christian Opitz, John Simon R Gibbs, Marion Delcroix, Da-Hee Park, Hossein Ardeschir Ghofrani, Ralf Ewert, Harald Kaemmerer, Hans-Joachim Kabitz, Dirk Skowasch, Juergen Behr, Katrin Milger, Tobias J Lange, Heinrike Wilkens, Hans-Jürgen Seyfarth, Matthias Held, Daniel Dumitrescu, Iraklis Tsangaris, Anton Vonk-Noordegraaf, Silvia Ulrich, Hans Klose, Martin Claussen, Stephan Eisenmann, Kai-Helge Schmidt, Andrew J Swift, Alfred A Roger Thompson, Charlie A Elliot, Stephan Rosenkranz, Robin Condliffe, David G Kiely, Michael Halank
• E-Jahr: 2022
• Jahr: October 2022
• Umfang: 12 S.
• Illustrationen: Graphen
• Fussnoten: Gesehen am 22.05.2023 ; Available online 28 June 2022, Version of Record 27 September 2022
• Schrift/Sprache: Mit einer Zusammenfassung in englischer Sprache
• Titel Quelle: Enthalten in: The lancet <London> / Respiratory medicine
• Ort Quelle: Oxford : Elsevier, 2013
• Jahr Quelle: 2022
• Band/Heft Quelle: 10(2022), 10, Seite 937-948
• ISSN Quelle: 2213-2619
• DOI: doi:10.1016/S2213-2600(22)00097-2
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1845938992

Abstract

Background - Among patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH), there is an emerging lung phenotype characterised by a low diffusion capacity for carbon monoxide (DLCO) and a smoking history. The present study aimed at a detailed characterisation of these patients. - Methods - We analysed data from two European pulmonary hypertension registries, COMPERA (launched in 2007) and ASPIRE (from 2001 onwards), to identify patients diagnosed with IPAH and a lung phenotype defined by a DLCO of less than 45% predicted and a smoking history. We compared patient characteristics, response to therapy, and survival of these patients to patients with classical IPAH (defined by the absence of cardiopulmonary comorbidities and a DLCO of 45% or more predicted) and patients with pulmonary hypertension due to lung disease (group 3 pulmonary hypertension). - Findings - The analysis included 128 (COMPERA) and 185 (ASPIRE) patients with classical IPAH, 268 (COMPERA) and 139 (ASPIRE) patients with IPAH and a lung phenotype, and 910 (COMPERA) and 375 (ASPIRE) patients with pulmonary hypertension due to lung disease. Most patients with IPAH and a lung phenotype had normal or near normal spirometry, a severe reduction in DLCO, with the majority having no or a mild degree of parenchymal lung involvement on chest computed tomography. Patients with IPAH and a lung phenotype (median age, 72 years [IQR 65-78] in COMPERA and 71 years [65-76] in ASPIRE) and patients with group 3 pulmonary hypertension (median age 71 years [65-77] in COMPERA and 69 years [63-74] in ASPIRE) were older than those with classical IPAH (median age, 45 years [32-60] in COMPERA and 52 years [38-64] in ASPIRE; p<0·0001 for IPAH with a lung phenotype vs classical IPAH in both registries). While 99 (77%) patients in COMPERA and 133 (72%) patients in ASPIRE with classical IPAH were female, there was a lower proportion of female patients in the IPAH and a lung phenotype cohort (95 [35%] COMPERA; 75 [54%] ASPIRE), which was similar to group 3 pulmonary hypertension (336 [37%] COMPERA; 148 [39%] ASPIRE]). Response to pulmonary arterial hypertension therapies at first follow-up was available from COMPERA. Improvements in WHO functional class were observed in 54% of patients with classical IPAH, 26% of patients with IPAH with a lung phenotype, and 22% of patients with group 3 pulmonary hypertension (p<0·0001 for classical IPAH vs IPAH and a lung phenotype, and p=0·194 for IPAH and a lung phenotype vs group 3 pulmonary hypertension); median improvements in 6 min walking distance were 63 m, 25 m, and 23 m for these cohorts respectively (p=0·0015 for classical IPAH vs IPAH and a lung phenotype, and p=0·64 for IPAH and a lung phenotype vs group 3 pulmonary hypertension), and median reductions in N-terminal-pro-brain-natriuretic-peptide were 58%, 27%, and 16% respectively (p=0·0043 for classical IPAH vs IPAH and a lung phenotype, and p=0·14 for IPAH and a lung phenotype vs group 3 pulmonary hypertension). In both registries, survival of patients with IPAH and a lung phenotype (1 year, 89% in COMPERA and 79% in ASPIRE; 5 years, 31% in COMPERA and 21% in ASPIRE) and group 3 pulmonary hypertension (1 year, 78% in COMPERA and 64% in ASPIRE; 5 years, 26% in COMPERA and 18% in ASPIRE) was worse than survival of patients with classical IPAH (1 year, 95% in COMPERA and 98% in ASPIRE; 5 years, 84% in COMPERA and 80% in ASPIRE; p<0·0001 for IPAH with a lung phenotype vs classical IPAH in both registries). - Interpretation - A cohort of patients meeting diagnostic criteria for IPAH with a distinct, presumably smoking-related form of pulmonary hypertension accompanied by a low DLCO, resemble patients with pulmonary hypertension due to lung disease rather than classical IPAH. These observations have pathogenetic, diagnostic, and therapeutic implications, which require further exploration. - Funding - COMPERA is funded by unrestricted grants from Acceleron, Bayer, GlaxoSmithKline, Janssen, and OMT. The ASPIRE Registry is supported by Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK.