TY - JOUR T1 - Comparison of clinical characteristics and prognostic factors between combined pulmonary fibrosis and emphysema/UIP versus non-UIP JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P3378 AU - Keishi Sugino AU - Fumiaki Ishida AU - Naoshi Kikuchi AU - Nao Hirota AU - Go Sano AU - Keita Sato AU - Kazutoshi Isobe AU - Susumu Sakamoto AU - Yujiro Takai AU - Sakae Homma Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P3378.abstract N2 - Aim: The aim of this study was to compare the clinical characteristics and the prognosis between combined pulmonary fibrosis and emphysema (CPFE) associated with usual interstitial pneumonia pattern (UIP) and CPFE with non-UIP on chest HRCT images.Patients and Methods: 34 patients were diagnosed as CPFE/UIP, 14 as CPFE/possible UIP, and 11 as CPFE/inconsistent with UIP according to the ATS/ERS/JRS/ALAT new IPF guideline in our institution. The HRCT scan imaging patterns of UIP pattern and possible UIP pattern were defined as UIP, and inconsistent with UIP pattern as non-UIP. We retrospectively compared the clinical features and prognosis between CPFE with UIP and CPFE with non-UIP.Results: CPFE/UIP had significantly an increase in fibrosis score compared with CPFE/non-UIP (p = 0.0218), whereas, no significant difference was found between 2 groups in emphysema score. Survival time was significantly lower in patients with CPFE/UIP than that in those with CPFE/non-UIP (p = 0.028). The univariate Cox regression model showed that the predictive factors were high esPAP, fibrosis score, and composite physiologic index, presence of honeycomb lung, low %FVC and %DLco, and acute exacerbation. We classified patients into 4 groups; UIP with high-esPAP (esPAP ≥ 34.6 mmHg), UIP with normal esPAP (esPAP < 34.6 mmHg), non-UIP with high-esPAP, and non-UIP with normal esPAP. The survival in UIP with high-esPAP had significantly worse than that in other 3 subgroups (p = 0.0190).Conclusions: Patients with CPFE/UIP have a worse prognosis than those with CPFE/non-UIP. Especially, patients with CPFE/UIP associated with high-esPAP have an extremely poor prognosis. ER -