TY - JOUR T1 - 'Idiopathic' pulmonary arterial hypertension with preserved lung function but co-existing parenchymal abnormalities: Response to treatment and survival JF - European Respiratory Journal JO - Eur Respir J VL - 40 IS - Suppl 56 SP - 371 AU - Yi Ling AU - Martin Johnson AU - David Kiely AU - Robin Condliffe AU - Charlie Elliot AU - J. Simon R Gibbs AU - Luke Howard AU - Joanna Pepke-Zaba AU - Karen Sheares AU - Paul Corris AU - Andrew Fisher AU - James Lordan AU - Sean Gaine AU - J. Gerry Coghlan AU - S. John Wort AU - Michael Gatzoulis AU - Andrew Peacock Y1 - 2012/09/01 UR - http://erj.ersjournals.com/content/40/Suppl_56/371.abstract N2 - Background:FEV1, FVC and/or TLC <60% were used in the French and Scottish registries to exclude patients with pulmonary hypertension (PH) due to lung disease. Similar criteria were used in pivotal PH trials to exclude significant lung disease. However, some patients with preserved pulmonary function who satisfy the standard criteria for idiopathic pulmonary arterial hypertension (IPAH) may nonetheless have evidence of co-existing parenchymal abnormalities on HRCT. It is unclear how response to treatment and survival is affected by the presence of modest lung disease.Aim:To compare the baseline characteristics, change in 6-minute walk distance at 3 months and survival of IPAH with and without co-existing parenchymal abnormalities on HRCT.Methods:All incident cases of IPAH with and without co-existing CT parenchymal abnormalities diagnosed between January 2001 to December 2009 in all eight PH centres in the UK and Ireland were included. All patients have FEV1, FVC and/or TLC≥ 60% predicted.Results:View this table:Table 1. Baseline characteristics, Δ6MWD and survival of IPAHConclusion:Despite similar baseline haemodynamics and response to treatment, survival of 'IPAH' with coexisting parenchymal abnormalities appears worse compared with IPAH without parenchymal abnormalities. Age and age related co-morbidities may account for the difference in long term outcome between the 2 groups. ER -