RT Journal Article
SR Electronic
T1 Can clinical data predict rapid decline of lung function in idiopathic pulmonary fibrosis (IPF) patients?
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA3821
DO 10.1183/1393003.congress-2017.PA3821
VO 50
IS suppl 61
A1 Veronika Müller
A1 Nesrin Mogulkoc
A1 Martina Šterclová
A1 Beata Zolnowska
A1 Vladimír Bartoš
A1 Martina Plačková
A1 Ladislav Lacina
A1 Robert Slivka
A1 Martina Doubková
A1 Marina Roksandić Milenkovic
A1 Radka Bittenglová
A1 Imrich Jonner
A1 Magdalena Martusewicz-Boros
A1 Monika Žurková
A1 Ilona Binková
A1 Małgorzata Sobiecka
A1 Richard Tyl
A1 Marzena Trzaska-Sobczak Marzena Trzaska-Sobczak
A1 Pawel Sliwinski
A1 Vladimíra Lošťáková
A1 Sebastian Majewski
A1 Pavlína Lisá
A1 Katarzyna Lewandowska
A1 Hana Šuldová
A1 Jasna Tekavec-Trkanje
A1 Amelia Szymanowska-Narloch
A1 Lenka Šišková
A1 Anikó Bohács
A1 Ján Plutinský
A1 Bohumil Matula
A1 Štefan Tóth
A1 František Petřík
A1 Bučeková Bučeková
A1 Jana Pšíkalová
A1 Miklós Zsiray
A1 Jaroslav Lněnička
A1 Vladimír Řihák
A1 Zsuzsanna Szalai
A1 Zoltan Baliko
A1 Štefan Laššán
A1 Pavlína Musilová
A1 Imre Lajko
A1 Tomáš Snížek
A1 Jan Anton
A1 Jiří Homolka
A1 Peter Palúch
A1 Róbert Vyšehradský
A1 Renata Králová
A1 Jan Kervitzer
A1 Maria Szilasi
A1 Jan Kus
A1 Dragana Jovanovic
A1 Marta Hájková
A1 Michal Svoboda
A1 Jana Strenková
A1 Martina Vašáková
A1 Daniel Doležal
YR 2017
UL http://erj.ersjournals.com/content/50/suppl_61/PA3821.abstract
AB Key feature of IPF is decline in forced vital capacity (FVC) and CO diffusion (DLco), but data on clinical determinants of this change in patients who have established disease are scarce. For a given patient it is unknown if different natural histories represent distinct phenotypes of IPF or if geographic, ethnic or other factors might have influence on progression.The EMPIRE (European Multipartner IPF Registry) is a multicountry IPF register in the CEE region. Out of 1229 patients from the registry 324 with available data on FVC and DLco at inclusion and at 6 months rapid decliners (RD: FVC >10% or DLco >10% in 6 months) and controls (C) were included into the analysis. Clinical characteristics at inclusion were analyzed between groups to find clinical determinants of RD.Average age was 67,4±9.7 years, more males were affected than females (male:female 221:103) most patients had classic signs of UIP on HRCT. Most common comorbidity was hypertension (50.9%), coronary heart disease (20.5%), diabetes (20.1%) and hyperlipidaemia (20.1%). RD of FVC was present in 33.7%, while RD of DLco in 53.3% of patients. Comparison of RD and C patients for smoking status, dyspnoe severity, comorbidities, HRCT pattern, lung function parameters at inclusion, 6 minute walk distance or the presence of pulmonary hypertension did not show any difference between groups.The rate of change in FVC or DLco among IPF patients is highly variable, with 34-53 % of patients showing increased rates of decline in FVC and/or DLco. No clinical characteristics could be identified which can predict rapid decline using the data of EMPIRE registry.