TY - JOUR T1 - Clinical course of idiopathic pulmonary fibrosis (IPF): Prediction and outcome JF - European Respiratory Journal JO - Eur Respir J VL - 38 IS - Suppl 55 SP - p648 AU - Corina Probst AU - Elena Bargagli AU - Paola Rottoli AU - Florian Kollert AU - Kerstin Höhne AU - Gernot Zissel AU - Joachim Müller-Quernheim AU - Antje Prasse Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/Suppl_55/p648.abstract N2 - 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. ER -