Mild parenchymal lung disease and/or low diffusion capacity impacts survival and treatment response in patients diagnosed with idiopathic pulmonary arterial hypertension.

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 IPAH

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Conflict of interest: R.A. Lewis reports non-financial support from Actelion Pharmaceuticals, outside the submitted work. Conflict of interest: A.A.R. Thompson reports grants from British Heart Foundation, during the conduct of the study; support for meeting attendance from Actelion Pharmaceuticals Ltd, outside the submitted work. Conflict of interest: C.G. Billings has nothing to disclose. Conflict of interest: A. Charalampopoulos reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, personal fees and non-financial support from Novartis, grants from Bayer and GSK, outside the submitted work. Conflict of interest: C.A. Elliot reports personal fees for advisory board work and lectures from Actelion Pharmaceuticals, GlaxoSmithKline and Bayer, grants from Pfizer, Actelion Pharmaceuticals and Bayer, support for meeting attendance from Bayer and Actelion Pharmaceuticals, outside the submitted work. Conflict of interest: N. Hamilton reports personal fees from MSD and Actelion, grants and personal fees from Bayer, outside the submitted work. Conflict of interest: C. Hill has nothing to disclose. Conflict of interest: J. Hurdman has nothing to disclose. Conflict of interest: S. Rajaram has nothing to disclose. Conflict of interest: I. Sabroe reports grants and personal fees for advisory board from AstraZeneca, grants from GSK, outside the submitted work. Conflict of interest: A.J. Swift has nothing to disclose. Conflict of interest: D.G. Kiely reports grants, personal fees and non-financial support from Actelion, Bayer and GSK, personal fees and non-financial support from MSD, outside the submitted work. Conflict of interest: R. Condliffe reports grants, personal fees and non-financial support from Actelion Pharmaceuticals and Bayer, grants from GSK, outside the submitted work.