作者
Robert A Lewis, AA Roger Thompson, Catherine G Billings, Athanasios Charalampopoulos, Charlie A Elliot, Neil Hamilton, Catherine Hill, Judith Hurdman, Smitha Rajaram, Ian Sabroe, Andy J Swift, David G Kiely, Robin Condliffe
发表日期
2020/6/1
期刊
European Respiratory Journal
卷号
55
期号
6
出版商
European Respiratory Society
简介
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).
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 IPAHno-LD (n=303), and those with minor/mild emphysema or fibrosis were described as IPAHmild-LD (n=190).
Survival was significantly better in IPAHno-LD than in IPAHmild-LD (1- and 5-year survival 95% and 70% versus 78% and 22%, respectively; p<0.0001). In the combined group of IPAHno-LD and IPAHmild-LD, independent predictors of higher mortality were …
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RA Lewis, AAR Thompson, CG Billings… - European Respiratory Journal, 2020