Results: The database comprises 3895 PBC patients of which 2924 U D C A-treated patients with available lab measurements; mean age of 52.3 (±12.2) yrs, female: 91%, AMA+: 88%. Median follow up time learn more was 7 (IQR 3-11) yrs. LTX-free survival was significantly better for patients responding to treatment as assessed by all of the models. Rotterdam and Paris I criteria were the most powerful predictors, hazard ratio (HR) respectively: 3.92 (3.17-4.85) and 4.25 (3.53-5.11) for non-responders versus responders. According to Rotterdam and Paris I criteria 10-yrs survival was 84.1 % and 88.1% for responders and 42.7% and 50.1% for nonresponders.

Cox regression analysis showed Barcelona, Paris I, Rotterdam and Toronto criteria were independently associated with LTX-free survival (c-statistics: 0.78 (0.74-0.81)). 38% of patients responded according to all criteria (10-yrs survival: 96.7%, sensitivity: 88.6%), while 10.4% did not respond according to any criteria (10-yrs survival: 58.0%, HR=7.7 (5.510.7)). Conclusions: This analysis of a large pooled UDCAtreated PBC cohort

confirms the prognostic value of previously proposed response criteria. Paris I and Rotterdam were the most powerful predictors. Four of the five criteria Rapamycin purchase contribute independently in a combined analysis of prognostic significance, suggesting that the optimal response criteria await to be defined. Barcelona (normal ALP or >40% decrease) Paris I (ALP≦ 3xULN, AST≦ 2xULN, normal bili) Rotterdam (normaliation of abnormal bili and/or albumin) Toronto (ALP<1.67xULN) Paris II (ALP≦ 1.5xULN, AST≦ 1.5xULN, normal bili) HR no response vs response (95% CI) 1.95 (1.62-2.34) Tau-protein kinase 4.25(3.53-5.11) 3.92 (3.17-4.85) 2.60(2.13-3.17) 2.99 (2.40-3.71) at 10 year sensitivity specificity PPV NPV 63% 59% 68% 53% 71% 72% 75% 69% 83% 59% 72% 73% 66% 60% 54% 71% 45% 84% 77% 57% c-statistics (95% CI) 0.69 (0.66-0.72) 0.76 (0.74-0.78) 0.74 (0.71-0.78) 0.71 (0.69-0.74) 0.71 (0.68-0.73) Disclosures: Gideon M. Hirschfield – Advisory Committees or Review Panels: Centocor/J&J, Medigene, Intercept, Falk Pharma; Consulting: Lumena,

Intercept Harry L. Janssen – Consulting: Abbott, Bristol Myers Squibb, Debio, Gilead Sciences, Merck, Medtronic, Novartis, Roche, Santaris; Grant/Research Support: Anadys, Bristol Myers Squibb, Gilead Sciences, Innogenetics, Kirin, Merck, Medtronic, Novartis, Roche, Santaris Cyriel Y. Ponsioen – Consulting: AbbVIE; Grant/Research Support: AbbVIE, Schering Plough, Dr. Falk Pharma, Tramedico Netherlands Marlyn J. Mayo – Consulting: Mitsubishi, Regeneron; Grant/Research Support: Intercept, Lumena Jayant A. Talwalkar – Consulting: Lumena; Grant/Research Support: Intercept, Salix, Gilead Frederik Nevens – Grant/Research Support: Ipsen, Roche, MSD, Astellas, CAF Andrew L. Mason – Grant/Research Support: Abbott, Gilead Kris V.