Increased serum levels of carbohydrate antigen 19-9 and outcomes in primary sclerosing cholangitis patients without cholangiocarcinoma.

BACKGROUND

Carbohydrate antigen 19-9 (CA 19-9) is the serum marker used to diagnose cholangiocarcinoma (CCA) in patients with primary sclerosing cholangitis (PSC).

OBJECTIVE

We investigated long-term outcomes in patients with PSC and elevated CA 19-9 levels without CCA.

METHODS

A total of 166 PSC patients with serum levels of CA 19-9 without CCA followed at a single center from 1998 to 2011 were included. Patients with and without elevated CA 19-9 levels (greater than 129 U/ml) were compared.

RESULTS

Fifty-two (31.3 %) of the 166 patients with PSC without CCA had elevated serum CA-19-9. Patients with elevated CA-19-9 were followed for a mean of 4 years and 12 (23.1 %) died. They were more likely to have higher PSC risk score (1.67 ± 1.30 vs. 0.91 ± 1.53, p = 0.003) and dominant strictures (31 (59.6 %) vs. 21 (18.4 %), p < 0.001). In 17/52 (32.7 %) of patients with elevated CA-19-9, no etiology was identified; cholestasis and cholangitis were associated with elevated levels in 24/52 (48.1 %) and 11/52 (21.2 %), respectively. There were 32 of 52 (62.5 %) that underwent orthotopic liver transplantation (OLT) in elevated CA 19-9 group compared to 66/114 (56.9 %) without (p = 0.66). The median OLT-free survival with elevated CA 19-9 was 9 years from PSC diagnosis compared to 14 years without. Although there was a trend, there was no significant difference in the OLT-free survival on Kaplan-Meier analysis (log rank p = 0.12).

CONCLUSIONS

Thirty-two percent of patients with PSC had elevated serum CA 19-9 in the absence of CCA. There was a trend towards shorter OLT-free survival in PSC patients with elevated CA-19-9.