printing supported by . Visit Chiesi at Stand D.30 SUNDAY, SEPTEMBER 25TH 2011 was related to respiratory causes in 78% (AE: 37% and end-stage respiratory insufficiency: 26%). Conclusion: Our results confirm the high frequency of overweight and severe comorbidities in IPF. Despite a relatively low incidence of evolutive events, mortality is already high, with AE being the major cause of death. P647 Arterial stiffness and endothelial dysfunction in idiopathic pulmonary fibrosis (IPF) Sergey Avdeev, Marina Makarova, Alexander Chuchalin. Clinical Department, Pulmonology Research Institute, Moscow, Russian Federation Background: Fibrotic lung diseases are associated with an increased prevalence of coronary artery disease and cardiovascular complications [Kizer et al., 2004]. Arterial stiffness and endothelial dysfunction are widely accepted as markers of cardiovascular risk. Objectives: The aim of the present study was to assess aortic stiffness and endothelial dysfunction in patients with IPF and to determine the association of these markers with other clinical and functional parameters. Methods: We enrolled 25 IPF patients (age 57±8 yrs; FVC 80±18%; DLCO 38±15%) and 30 normal control subjects (age 52±5 yrs). Assessment of arterial stiffness was performed by use of digital photoplethysmography (Pulse Trace PCA 2, Micro Medical). Change in reflection index of the digital volume pulse in response to salbutamol ( RISALB) was used to assess endothelial function. Results: In IPF patients stiffness index (SI) was significantly higher than in normal control subjects: 9.8±3.1 vs 6.9±1.0 m/s (p<0.001). The correlations between SI and sleep time spent with SpO2 < 88% (r=-0.67, p<0.05) and total serum cholesterol level (r=-0.77, p<0.05) were highly significant in IPF patients. RISALB was significantly lower in IPF patients than in control subjects: 2.2±1.2% vs 15.8±9.3% (p=0.01). RISALB was significantly associated with FEV1 (r=0.57, p<0.05), mean nocturnal SpO2 (r=-0.83, p<0.05) and total cholesterol level (r=-0.71, p<0.05). Conclusions: Arterial stiffness and endothelial dysfunction are significantly impaired in IPF patients. Decreased FEV1 was associated with endothelial dysfunction. Nocturnal hypoxemia and total cholesterol level have an association with both arterial stiffness and endothelial function. P648 Clinical course of idiopathic pulmonary fibrosis (IPF): Prediction and outcome Corina Probst1, Elena Bargagli2, Paola Rottoli2 , Florian Kollert3 , Kerstin Höhne1, Gernot Zissel1, Joachim Müller-Quernheim1, Antje Prasse1. 1Pneumology, University Medical Centre Freiburg, Freiburg, Germany; 2Rheumatology, University Medical Centre Freiburg, Freiburg, Germany; 3Department of Clinical Medicine and Immunological Sciences, Siena University, Siena, Italy Background: IPF is a progressive disease for which a median survival time of 2.8 years was reported. However the clinical course of IPF is variable. Acute exacerbation (AE) is a major cause of death in IPF, but only a minority of patients develop AE. Objectives: The aim of this study was to examine different clinical courses of IPF and to evaluate associated risk factors and predictors. Methods: We retrospectively studied 85 consecutive patients diagnosed with IPF based on the criteria of the ATS/ERS consensus statement. Clinical data and serial pulmonary function tests were obtained. In accordance to King et al. (King, TE et al. AJRCCM 2011; 183:431-40) patients were grouped to four clinical phenotypes: stable disease, slowly progressive, rapid progressive to death or mixed course. Furthermore, AEs as defined by the criteria of Collard et al. (Collard, HD et al. AJRCCM 2007; 176:636-643) were reported. Serial serum CCL18 concentrations were measured by ELISA. Results: AE occurred in 34 (40%) patients, with multiple episodes in 6 (7%) patients. The 5-yr survival rate of IPF patients with and without AE was 15% and 41%. Baseline CCL18 serum concentrations differed significantly between the four clinical phenotypes (p<0.0001). IPF patients with a rapid progressive or mixed course showed higher CCL18 serum concentrations (p<0.0001), lower FVC predicted (FVC<60%; p=0.0156) and lower TLC predicted (p=0.002). Stepwise multivariate regression analysis of all patients revealed that CCL18 serum concentration (p=0.016) was independently associated with AE. Conclusions: We demonstrate that baseline serum CCL18 levels are elevated in IPF patients prone to AE and predict a rapid progressive or mixed course of IPF. P649 Risk factors of acute exacerbation of idiopathic pulmonary fibrosis-extended analysis of the pirfenidone trial in Japan Hiroyuki Taniguchi1, Yasuhiro Kondoh1, Masahito Ebina2, Arata Azuma3, Takashi Ogura4, Moritaka Suga5, Yoshio Taguchi6, Hiroki Takahashi7, Koichiro Nakata8, Atuhiko Sato9, Yukihiko Sugiyama10, Shoji Kudoh3, Toshihiro Nukiwa2. 1Dept. of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan; 2Dept. of Respiratory Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan; 3Dept. of Internal Medicine, Nippon Medical School, Tokyo, Japan;4Dept. of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan; 5Dept. of Respiratory Medicine, Saiseikai Kumamoto Hospital, Kumamoto, Japan; 6Dept. of Respiratory Medicine, Tenri Hospital, Tenri, Japan; 7Third Dept. of Internal Medicine, Sapporo Medical University of Medicine, Sapporo, Japan; 8Dept. of Respiratory Medicine, Nakata Clinic, Tokyo, Japan; 9Dept. of Respiratory Medicine, Medicine Kyoto Preventive, Kyoto, Japan; 10Division of Pulmonary Medicine, Dept. of Medicine, Jichi Medical University, Tochigi, Japan Background:Although acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is a well known clinical condition, predicting risk factors remain unknown. Recent studies reported that various baseline factors, and at least 10% decline in FVC at six months were reported as risk factor for AE (Sarcoidosis Vasc Diffuse Lung Dis 2010;27:103-110). We sought to evaluate the risk factors of AE by analyzing our phase III clinical trial of pirfenidone for patients with IPF (n=275) (Eur Respir J 2010; 35: 821-9). Methods: Baseline characteristics including age, sex, smoking, BMI, dyspnoea grade (Hugh-Jones classification), AaDO2, PaO2,%VC,%DLco, KL-6, SP-A, SPD were evaluated as possible risk factors for AE. Decline of VC≥10% within 6 months was also evaluated. In addition, effect of pirfenidone therapy was also evaluated. Results: During 52 weeks, 14 patients experienced AE-IPF. Univariate analysis by Cox proportion hazards model were as follows: age (HR, 0.982, p=0.642), sex (HR, 1.505, p=0.489) smoking (HR, 0.464, p=0.168), BMI (HR, 0.935, p=0.460), dyspnea grade (HR, 1.763, p=0.168), AaDO2 (HR, 1.047, p=0.069), PaO2 (HR, 0.955, p=0.110),%VC (HR, 0.971, p=0.078),%DLco (HR, 0.994, p=0.714), KL-6 (HR, 1.000, p=0.698), SP-A (HR, 0.998, p=0.776), SP-D (HR, 1.000, p=0.875), pirfenidone treatment (HR, 1.211, p=0.732). Decline of VC was a significant risk factor for AE-IPF (HR, 3.780, p=0.014). Stepwised multivariate analysis revealed that initial AaDO2 (HR, 1.055, p=0.045) and decline in VC within 6 months (HR, 3.951, p=0.012) were significant risk factors for AE-IPF. Conclusions: Baseline higher AaDO2 and decline of VC≥10% within 6 months are significant risk factors of AE-IPF. P650 Clinical profile of idiopathic pulmonary fibrosis with lung cancer Christian Gurioli1 , Sara Tomassetti1, Claudia Ravaglia1, Micaela Romagnoli1, Gianluca Casoni1, Nicola Sverzellati2, Maurizio Zompatori3 , Marco Chilosi4, Venerino Poletti1 . 1Pulmonology Unit, Morgagni-Pierantoni Hospital, Forli’, Italy; 2Department of Clinical Sciences, Division of Radiology, University of Parma, Parma, Italy; 3Department of Radiology, S.Orsola Hospital of Bologna, University of Bologna, Bologna, Italy; 4Department of Pathology, University of Verona, Policlinico G. B. Rossi, Verona, Italy Background: The association of lung cancer (LC) and idiopathic pulmonary fibrosis (IPF) is well recognized. The poor prognosis of both conditions and the risk of serious complications related to LC treatment make the clinical management particularly difficult. Aims: To describe the clinical profile and survival of IPF associated or not with LC Methods: Retrospective cohort study from data collected in a prospective manner and retrieved from the IPF registry of Pulmonology Unit (Morgagni Hospital, Forlì, Italy). Study period from January 2000 to December 2010. Results: 208 patients diagnosed with IPF according to ATS/ERS criteria and followed in our IPF Clinic. Nineteen (9.1%) patients developed LC (cancer group, CG). Compared to patients without LC (non-cancer group, NCG), CG were older (68.5±7.9 vs 63.9±8.9 years), more frequently smokers (79.5% vs 67.8%) and heavier smokers (pky 44.3±32.1 vs 30.6±21.9), they had better baseline functional respiratory tests and similar number of familial IPF cases. There was not a statistically significant difference in median survival in the two groups (32 months in CG and 46 months in NCG, p=0.179). Conclusions: Cigarette smoke was the only clinical variable associated with increased risk of developing LC in IPF patients. We did not observe any difference in incidence of LC when comparing familial and sporadic forms of IPF. Survival did not differ despite better functional tests at baseline in the CG. 101s Thematic Poster Session Hall 2-8 12:50-14:40 Abstract printing supported by . Visit Chiesi at Stand D.30printing supported by . Visit Chiesi at Stand D.30 SUNDAY, SEPTEMBER 25TH 2011 P651 Effects of nitric oxide synthase up-regulation in early remodeling of usual interstitial pneumonia has impact on long term outcome of patients Edwin Roger Parra, Armando Aguiar Junior, Sandra Fernezlian, Vera Capelozzi. Pathology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil Recently, impaired endothelial-dependent vascular tone suggest that NOS enzymatic ac

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