Late Breaking Abstract - Survival in Idiopathic Pulmonary Fibrosis and Connective Tissue Disease-Interstitial Lung Disease

<bold>Aim:</bold> Compare survival between idiopathic pulmonary fibrosis-usual interstitial pneumonia (IPF-UIP) and connective tissue disease-interstitial lung disease (CTD–ILD) patients. <bold>Methods:</bold> Patients were typical 73 IPF-UIP (F 45, mean age 64.3 yrs, 28 smokers) and 33 CTD-ILD with systemic sclerosis (F19, mean age 51.4 yrs, 17 smokers). Physical examination, HRCT score, echocardiography for pulmonary artery pressure (PAP), New York Heart Association (NYHA) class, 6-minute walk test (6-MWT), DLCO, and Octreoscan were performed in all patients. Biopsy was done in CTD-ILD. Prednisone (PDN) and cyclophosphamide therapy were recorded. Survival was determined by Kaplan-Meier method. Odds of prognostic factors adjusted for age were calculated with multivariable logistic regression. <bold>Results:</bold> Median follow-up was 49.7 months (range 3.5 – 126.3). Median survival was 48.7 and 99.4 months in IPF-UIP (53 dead) and CTD-ILD (9 dead), respectively (p<0.001). Significant prognostic factors are reported in the Table. HRCT score was 4 in 100% IPF-UIP and 1, 2, and 3 in 27.3%, 30.3%, and 42.4%, respectively, in CTD-ILD. Mean Octreoscan Uptake Index (UI) was 9.5 and 12.6 in IPF-UIP and CTD-ILD, respectively (p<0.001). Survival was shorter in CTD-ILD patients treated with PDN than in those treated with PDN and cyclophosphamide. <bold>Conclusions:</bold> Survival was significantly shorter in IPF-UIP than in CTD-ILD and negatively associated with NYHA class and PAP. Octreoscan UI indicated inflammatory infiltrate in CTD-ILD. <table-wrap><object-id>erj;64/suppl_68/PA708/TB1</object-id><object-id>T1</object-id><object-id>TB1</object-id><table><colgroup><col></col><col></col><col></col><col></col><col></col></colgroup><tbody><tr><td>Prognostic Factor</td><td>IPF-UIP</td><td>IPF-UIP</td><td>CTD-ILD</td><td>CTD-ILD</td></tr><tr><td></td><td>OR</td><td>95% CI</td><td>OR</td><td>95% CI</td></tr><tr><td>NYHA class</td><td>3.9</td><td>2.5-6.2</td><td>6.6</td><td>1.8-23.4</td></tr><tr><td>PAP</td><td>1.2</td><td>1.1-1.3</td><td>1.1</td><td>1.0-1.2</td></tr><tr><td>6-MWT</td><td>0.98</td><td>0.97-0.99</td><td>0.96</td><td>0.93-0.99</td></tr><tr><td>DLCO</td><td>0.90</td><td>0.87-0.93</td><td>0.69</td><td>0.53-0.89</td></tr></tbody></table></table-wrap>