malignancy is 8-fold higher in PSC-IBD patients compared to those with IBD alone with up to 25-fold higher frequency of hepatobiliary cancers that include hepatocellular carcinomas. Hepatobiliary cancer screening in patients with PSC-IBD can be recommended. 1. Claessen M, Lutgens M, van Buuren H, Oldenburg B, Stokkers PC, van der Woude C, et al. More right-sided IBD-associated colorectal cancer in patients with primary sclerosing cholangitis. Inflamm Bowel Dis. 2009;15:1331–1336. MK SANDHU, W CUI, AE BLOCH, DM ISER, T NGUYEN, M RYAN, R CHEN, B DEMEDIUK, RG SHAW, SJ BELL, PV DESMOND, AJ THOMPSON St Vincent’s Hospital Melbourne, Victoria, Australia Background: Diagnostic criteria for PBC include elevated
serum ALP and positive AMA (titer ≥1:40). Gefitinib nmr PBC is associated with progressive liver disease, but this may be prevented in responders to ursodeoxycholic acid (UDCA). The clinical significance of positive AMA serology with normal ALP is not clear. There are limited Australian data describing the natural history of PBC and no Australia data for patients with positive AMA serology but normal ALP. We therefore performed a retrospective Cabozantinib manufacturer analysis of the clinical features and outcomes of patients with positive AMA serology over a 10 year period at a large tertiary referral center. Methods: Patients were identified through hospital pathology (AMA) and pharmacy claims data (UDCA) between 2003 and 2013. The diagnosis of PBC was made in the setting of an elevated ALP and a positive AMA (titer ≥1:40). We performed a cross-sectional
comparison of the clinical characteristics of patients selleck kinase inhibitor with PBC vs. patients with positive AMA serology not meeting diagnostic criteria for PBC. Among patients with longitudinal data, we evaluated clinical outcome with a specific focus on the impact of therapy with UDCA. In patients meeting the criteria for PBC, treatment response was defined by >40% reduction, or normalization, of ALP at 1 year (Barcelona criteria). Results: 72 patients with positive AMA serology were identified. 33/72 (46%) patients met the diagnostic criteria for PBC, including 3 patients with PBC-AIH overlap. 7 patients had positive AMA, normal ALP, but characteristic histology and were classified as PBC for this analysis. 32/72 (44%) patients had positive AMA serology but normal ALP; 4 patients in this group had positive HCV serology. The majority of patients were female and Caucasian (>80%); PBC patients were older and more likely to have advanced liver fibrosis, but there were no other significant differences between the groups. Longitudinal follow-up was available for the majority (63 [88%], median duration 60 [24–120] months). 9 PBC patients were lost to follow-up after diagnosis. 29/31(94%) patients with PBC were treated with UDCA. 2 PBC patients did not receive UDCA – both presented with advanced disease and were palliated.