Universität zu Köln

Hoeper, Marius M., Dwivedi, Krit, Pausch, Christine, Lewis, Robert A., Olsson, Karen M., Huscher, Doerte, Pittrow, David, Gruenig, Ekkehard, Staehler, Gerd, Vizza, Carmine Dario ORCID: 0000-0002-3540-4983, Gall, Henning, Distler, Oliver, Opitz, Christian, Gibbs, John Simon R., Delcroix, Marion, Park, Da-Hee ORCID: 0000-0003-0902-4773, Ghofrani, Hossein Ardeschir, Ewert, Ralf, Kaemmerer, Harald, Kabitz, Hans-Joachim, Skowasch, Dirk, Behr, Juergen, Milger, Katrin, Lange, Tobias J., Wilkens, Heinrike, Seyfarth, Hans-Juergen, Held, Matthias, Dumitrescu, Daniel, Tsangaris, Iraklis, Vonk-Noordegraaf, Anton, Ulrich, Silvia ORCID: 0000-0002-5250-5022, Klose, Hans, Claussen, Martin, Eisenmann, Stephan ORCID: 0000-0002-0387-0760, Schmidt, Kai-Helge, Swift, Andrew J., Thompson, Alfred A. Roger, Elliot, Charlie A., Rosenkranz, Stephan, Condliffe, Robin, Kiely, David G. and Halank, Michael (2022). Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis. Lancet Resp. Med., 10 (10). S. 937 - 949. OXFORD: ELSEVIER SCI LTD. ISSN 2213-2600

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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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Hoeper, Marius M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Dwivedi, Krit UNSPECIFIED UNSPECIFIED UNSPECIFIED Pausch, Christine UNSPECIFIED UNSPECIFIED UNSPECIFIED Lewis, Robert A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Olsson, Karen M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Huscher, Doerte UNSPECIFIED UNSPECIFIED UNSPECIFIED Pittrow, David UNSPECIFIED UNSPECIFIED UNSPECIFIED Gruenig, Ekkehard UNSPECIFIED UNSPECIFIED UNSPECIFIED Staehler, Gerd UNSPECIFIED UNSPECIFIED UNSPECIFIED Vizza, Carmine Dario UNSPECIFIED orcid.org/0000-0002-3540-4983 UNSPECIFIED Gall, Henning UNSPECIFIED UNSPECIFIED UNSPECIFIED Distler, Oliver UNSPECIFIED UNSPECIFIED UNSPECIFIED Opitz, Christian UNSPECIFIED UNSPECIFIED UNSPECIFIED Gibbs, John Simon R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Delcroix, Marion UNSPECIFIED UNSPECIFIED UNSPECIFIED Park, Da-Hee UNSPECIFIED orcid.org/0000-0003-0902-4773 UNSPECIFIED Ghofrani, Hossein Ardeschir UNSPECIFIED UNSPECIFIED UNSPECIFIED Ewert, Ralf UNSPECIFIED UNSPECIFIED UNSPECIFIED Kaemmerer, Harald UNSPECIFIED UNSPECIFIED UNSPECIFIED Kabitz, Hans-Joachim UNSPECIFIED UNSPECIFIED UNSPECIFIED Skowasch, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED Behr, Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Milger, Katrin UNSPECIFIED UNSPECIFIED UNSPECIFIED Lange, Tobias J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wilkens, Heinrike UNSPECIFIED UNSPECIFIED UNSPECIFIED Seyfarth, Hans-Juergen UNSPECIFIED UNSPECIFIED UNSPECIFIED Held, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED Dumitrescu, Daniel UNSPECIFIED UNSPECIFIED UNSPECIFIED Tsangaris, Iraklis UNSPECIFIED UNSPECIFIED UNSPECIFIED Vonk-Noordegraaf, Anton UNSPECIFIED UNSPECIFIED UNSPECIFIED Ulrich, Silvia UNSPECIFIED orcid.org/0000-0002-5250-5022 UNSPECIFIED Klose, Hans UNSPECIFIED UNSPECIFIED UNSPECIFIED Claussen, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Eisenmann, Stephan UNSPECIFIED orcid.org/0000-0002-0387-0760 UNSPECIFIED Schmidt, Kai-Helge UNSPECIFIED UNSPECIFIED UNSPECIFIED Swift, Andrew J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Thompson, Alfred A. Roger UNSPECIFIED UNSPECIFIED UNSPECIFIED Elliot, Charlie A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Rosenkranz, Stephan UNSPECIFIED UNSPECIFIED UNSPECIFIED Condliffe, Robin UNSPECIFIED UNSPECIFIED UNSPECIFIED Kiely, David G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Halank, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-698196
DOI:	10.1016/S2213-2600(22)00097-2
Journal or Publication Title:	Lancet Resp. Med.
Volume:	10
Number:	10
Page Range:	S. 937 - 949
Date:	2022
Publisher:	ELSEVIER SCI LTD
Place of Publication:	OXFORD
ISSN:	2213-2600
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language DIFFUSION CAPACITY; TOBACCO-SMOKE; THERAPY; AMBRISENTAN; TADALAFIL; MORTALITY; SURVIVAL Multiple languages Critical Care Medicine; Respiratory System Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/69819