{"id":22392,"date":"2022-09-10T05:06:00","date_gmt":"2022-09-09T21:06:00","guid":{"rendered":"http:\/\/csccm.org.cn\/?p=22392"},"modified":"2022-09-10T09:34:08","modified_gmt":"2022-09-10T01:34:08","slug":"lancet-respir-med%e5%8f%91%e8%a1%a8%e8%ae%ba%e6%96%87%ef%bc%9a%e7%89%b9%e5%8f%91%e6%80%a7%e8%82%ba%e5%8a%a8%e8%84%89%e9%ab%98%e5%8e%8b%e7%9a%84%e8%a1%a8%e5%9e%8b","status":"publish","type":"post","link":"https:\/\/csccm.org.cn\/?p=22392","title":{"rendered":"[Lancet Respir Med\u53d1\u8868\u8bba\u6587]\uff1a\u7279\u53d1\u6027\u80ba\u52a8\u8109\u9ad8\u538b\u7684\u8868\u578b"},"content":{"rendered":"\n

ARTICLES|ONLINE FIRST<\/a><\/p>\n\n\n\n

Phenotyping of idiopathic pulmonary arterial hypertension: a registry analysis<\/h1>\n\n\n\n

Marius M Hoeper, Krit Dwivedi, Christine Pausch, et al.<\/h3>\n\n\n\n

Lancet Respir Med Published:June 28, 2022<\/a><\/h3>\n\n\n\n

DOI:https:\/\/doi.org\/10.1016\/S2213-2600(22)00097-2<\/a><\/h3>\n\n\n\n

Summary<\/h2>\n\n\n\n

Background<\/h3>\n\n\n\n

Among patients meeting diagnostic criteria for idiopathic pulmonary arterial hypertension (IPAH), there is an emerging lung phenotype characterised by a low diffusion capacity<\/a> for carbon monoxide<\/a> (DLCO) and a smoking history. The present study aimed at a detailed characterisation of these patients.<\/p>\n\n\n\n

Methods<\/h3>\n\n\n\n

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<\/a> 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<\/a> (group 3 pulmonary hypertension).<\/p>\n\n\n\n

Findings<\/h3>\n\n\n\n

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>, a severe reduction in DLCO, with the majority having no or a mild degree of parenchymal lung involvement on chest computed tomography<\/a>. Patients with IPAH and a lung phenotype (median age, 72 years [IQR 65\u201378] in COMPERA and 71 years [65\u201376] in ASPIRE) and patients with group 3 pulmonary hypertension (median age 71 years [65\u201377] in COMPERA and 69 years [63\u201374] in ASPIRE) were older than those with classical IPAH (median age, 45 years [32\u201360] in COMPERA and 52 years [38\u201364] in ASPIRE; p<0\u00b70001 for IPAH with a lung phenotype vs<\/em> 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\u00b70001 for classical IPAH vs<\/em>IPAH and a lung phenotype, and p=0\u00b7194 for IPAH and a lung phenotype vs<\/em> 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\u00b70015 for classical IPAH vs<\/em>IPAH and a lung phenotype, and p=0\u00b764 for IPAH and a lung phenotype vs<\/em> group 3 pulmonary hypertension), and median reductions in N-terminal-pro-brain-natriuretic-peptide were 58%, 27%, and 16% respectively (p=0\u00b70043 for classical IPAH vs<\/em> IPAH and a lung phenotype, and p=0\u00b714 for IPAH and a lung phenotype vs<\/em>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\u00b70001 for IPAH with a lung phenotype vs<\/em> classical IPAH in both registries).<\/p>\n\n\n\n

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Interpretation<\/h3>\n\n\n\n

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.<\/p>\n\n\n\n

Funding<\/h3>\n\n\n\n

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.<\/p>\n","protected":false},"excerpt":{"rendered":"

ARTICLES|ONLINE FIRST Phenotyping of idiopathic pulmona […]<\/p>\n","protected":false},"author":3,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[32,23],"tags":[],"_links":{"self":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts\/22392"}],"collection":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=22392"}],"version-history":[{"count":1,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts\/22392\/revisions"}],"predecessor-version":[{"id":22393,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=\/wp\/v2\/posts\/22392\/revisions\/22393"}],"wp:attachment":[{"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=22392"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=22392"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/csccm.org.cn\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=22392"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}