RT Journal Article
SR Electronic
T1 Mild parenchymal lung disease and/or low diffusion capacity impacts survival and treatment response in patients diagnosed with idiopathic pulmonary arterial hypertension
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 2000041
DO 10.1183/13993003.00041-2020
A1 Robert A. Lewis
A1 A.A. Roger Thompson
A1 Catherine G. Billings
A1 Athanasios Charalampopoulos
A1 Charlie A. Elliot
A1 Neil Hamilton
A1 Catherine Hill
A1 Judith Hurdman
A1 Smitha Rajaram
A1 Ian Sabroe
A1 Andy J. Swift
A1 David G. Kiely
A1 Robin Condliffe
YR 2020
UL http://erj.ersjournals.com/content/early/2020/02/20/13993003.00041-2020.abstract
AB 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 2001–19 were identified in the ASPIRE registry. Using pre-specified criteria based on 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 increasing age, lower DLCO, lower exercise capacity and a diagnosis of IPAHmild-LD (p all <0.05). Exercise capacity and quality of life improved (p both <0.0001) following treatment in patients with IPAHno-LD but not IPAHmild-LD. A proportion of patients with IPAHno-LD had a DLCO <45%; these patients had poorer survival than patients with DLCO ≥45% although demonstrated improved exercise capacity following treatment.The presence of even mild parenchymal lung disease in patients who would be classified as IPAH according to current recommendations has a significant adverse effect on outcomes. This phenotype can be identified using lung function testing and clinical CT reports. Patients with IPAH, no lung disease and severely reduced DLCO may represent a further distinct phenotype. These data suggest that RCTs of targeted therapies in patients with these phenotypes are required.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Lewis reports non-financial support from Actelion Pharmaceuticals, outside the submitted work.Conflict of interest: Dr. Thompson reports grants from British Heart Foundation, during the conduct of the study; non-financial support from Actelion Pharmaceuticals Ltd, outside the submitted work.Conflict of interest: Dr. Billings has nothing to disclose.Conflict of interest: Dr. Charalampopoulos reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, personal fees and non-financial support from Novartis, grants from Bayer, grants from GSK, outside the submitted work.Conflict of interest: Dr. Elliot reports personal fees from Actelion Pharmaceuticals, personal fees from Glaxo SmithKline, grants from Pfizer, grants from Actelion Pharmaceuticals, grants from Bayer, grants from Bayer, grants from Actelion Pharmaceuticals, personal fees from Bayer, outside the submitted work.Conflict of interest: Dr. Hamilton reports personal fees from MSD, grants and personal fees from Bayer, personal fees from Actelion, outside the submitted work.Conflict of interest: Dr. Hill has nothing to disclose.Conflict of interest: Dr. Hurdman has nothing to disclose.Conflict of interest: Dr. Rajaram has nothing to disclose.Conflict of interest: Dr. Sabroe reports grants and personal fees from Astra Zeneca, grants from GSK, outside the submitted work.Conflict of interest: Dr. Swift has nothing to disclose.Conflict of interest: Dr. Kiely reports grants, personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Bayer, grants, personal fees and non-financial support from GSK, personal fees and non-financial support from MSD, outside the submitted work.Conflict of interest: Dr. Condliffe reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, grants, personal fees and non-financial support from Bayer, grants from GSK, outside the submitted work.